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Annotations MSAB Database The HSF MSAB Peer-Reviewed Database with Annotations: encouraging and hastening research into Hidradenitis Suppurativa A considerable amount of Hidradenitis Suppurativa research has been conducted over the years, yet many of these documents are not easily accessible to either medical professionals or patients. By increasing the availability of past and current medical research, the HSF seeks to improve the quality of life for people with HS, enhance the quality of medical care they receive, and raise the profile and understanding of this disease on a global basis. By providing this resource, the HSF intends to encourage and hasten research. This database contains historical medical research that is selected, reviewed, categorized and annotated by the HSF Medical and Scientific Advisory Board from December 2005 and completed in May 2006. A content list is included within the Annotation PDF by file name, document name and year of publication. These documents have been published in peer-reviewed medical and scientific journals. All documents are in English. It costs time, effort and money to provide such valuable and accurate health information on HS. That is why we ask for your financial support of the mission, vision and programs of the HSF. Please visit our donate page and help us help those with HS: http://www.hs-foundation.org/support/donate.htm The "HSF MSAB Peer-Reviewed Database" specifically targets the treating physician and skin researchers. Doctors who treat HS can use this resource to aid their treatment of those with HS. They need to know what they can tell the patient; which clinical and laboratory examinations are required; which treatments must be discussed; and what the preferred options are. Researchers, the medical and pharmaceutical industries, and both government health and medical insurance authorities can use this resource in a multitude of ways, from elucidating the clinical phenotype to reviewing past medical research; to researching the etiology, morbidity and epidemiology of HS; to researching emerging treatments. This tool should eventually allow all concerned to be able to define the condition; separate evidence and the possible from coincidence and the impossible; set the best directions for research; and evaluate which emerging treatments for HS/AI are good candidates for further study and possess the potential to be developed by industry. Literature reviews of Hidradenitis Suppurativa (HS)/Acne Inversa are problematic because of recurring misunderstandings of terminology; numerous ill-defined therapeutic interventions; inaccurate descriptions of lesions; and even conflicting definitions of the disorder itself. Disease reviews and health information on the internet has sometimes been written without the authors having access to the best evidence, or the most current evidence, or the expertise to assess that evidence. Of over 450 citations on PubMed for Acne Inversa and Hidradenitis Suppurativa there are links to only 17 full text papers available without charge. Patients have reported that they have searched for decades without finding information that could help explain the nature of this complex disease, its morbidity, associated complications and treatments. Physicians and researchers continually express frustration at their inability to access many of the HS publications. The timely provision of accurate and reliable HS-specific information through the HSF MSAB Peer-Reviewed Database will encourage and hasten research into more effective therapies and, one hopes, an eventual cure for this disease. The HSF FTP research global database (1) was created in April 2005 by two patients with this disease in order to provide a refereed resource to doctors and patients and to satisfy what they saw as the very basic and previously unmet need of patients (and their doctors), to understand HS. Michelle Barlow, president of the HSF, states "Robert Howes (HSF vice president) and I wanted to define the problems with reference to past research in order to actively provide solutions; to improve patients’ quality of life; and to enhance the quality of medical care we receive." This one-stop research database, by virtue of its continual growth and adaptation to suit industry, research, physician and patient needs, has the potential to coordinate and change for the better how research is performed on this disease, and in addition will certainly improve the quality of information provided publicly to patients. It is a work in progress and your comments, suggestions and contributions are solicited. As physicians and scientists who have volunteered time, money and resources towards providing for the health and well-being of those affected by this disease, we hope to breathe life into this source of educational material for 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 1 Annotations MSAB Database Hidradenitis Suppurativa sufferers and their physicians. The Board has selected these landmark peer-reviewed items with the aim of making them freely available (2). Signed, Richard L. Gallo (United States) Gregor BE. Jemec (Denmark) Ichiro Kurokawa (Japan) Lynette J. Margesson (United States) W.H. Irwin McLean (United Kingdom) Samuel L. Moschella (United States) Ralf Paus (Germany) Jean Revuz (France) Rodney Sinclair (Australia) Jan von der Werth (United Kingdom) Christos C. Zouboulis (Germany) The Medical and Scientific Advisory Board, Hidradenitis Suppurativa Foundation, Inc. Notes: 1. Hidradenitis Suppurativa Foundation, Inc. HSF FTP Hidradenitis Suppurativa Research global Database.<http://hsfoundation.org/>. Accessed 3/18/2006. Hidradenitis Suppurativa Foundation, Inc., San Diego, United States., 2005 Apr. 2. Copyright Disclaimer Copyright laws may restrict some of the articles on the FTP site. These articles are provided by the HSF for their exclusive use in teaching, scholarship and research regarding Hidradenitis Suppurativa. To the best of our knowledge, in supplying this material to you, we have followed the United States guidelines of Title 17, Chapter 1, Section 107 regarding fair use of copyright materials. 3. MSAB Annotated Database Folder categories: MSAB ANNOTATIONS.rtf 1 Recommended Reading 2 Prevalence_Morbidity_Epidemiology 3 Co-morbidity_Complications 4 Pathogenesis_Cellular_Molecular_Genetic 5 Co-morbid Bone and Joint Disease 6 Differential & Missed Diagnosis 7 Immunity Inflammation 8 Hormones 9 Surgery 10 Other treatments HSF MSAB Peer-Reviewed Database with Annotations (Referenced by year of publication). 1 Recommended Reading: 2006 Abstracts of HSF Symposium Experimental Dermatology HSF.pdf Hidradenitis Suppurativa Foundation (2006) Abstracts from Directions 2006: The First International Hidradenitis Suppurativa Research Symposium, Dessau, Germany, March 30-April 2, 2006 Experimental Dermatology 15 (6), 478482. doi: 10.1111/j.0906-6705.2006.0436a.x 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 2 Annotations MSAB Database The Hidradenitis Suppurativa Foundation, Inc. (HSF) is a non-profit organization based in San Diego, California whose mission is to improve the quality of life and quality of care for individuals and families affected by Hidradenitis Suppurativa (HS), also called Acne Inversa. Forging international and cooperative research efforts, a group of doctors and scientists met in Dessau, Germany for "Directions 2006," the First International HS Research Symposium, from March 30 to April 2, 2006. This event was organized by the HSF, the Dessau Medical Center and the EADV Task Force on “Acne and Rosacea.” The abstracts provide essential, up-to-date public health information and detail new concepts and research findings on HS pathogenesis and management. 2006 Teach me and I will be silent Seton.pdf Seton M. "Teach me, and I will be silent; make me understand how I have erred." (job 6:24). Ann Intern Med. 2006 Mar; 144(6):449-450. Compassionate essay from a doctor caring for a man suffering from hidradenitis suppurativa, polyarthritis and pyoderma gangrenosum. 2004 Review Histopathology HS is AI Sellheyer.pdf Sellheyer K, Krahl D. "Hidradenitis suppurativa" is acne inversa! An appeal to (finally) abandon a misnomer. Online Early, International Journal of Dermatology 0 (0) <http://www.blackwellsynergy.com/links/doi/10.1111/j.13654632.2004.02536.x/full/>. Accessed 4/24/2005. Blackwell Publishing, UNITED STATES, 2004. combines pathology from skin samples and the history of HS to define disease This historical review of pathogenesis also includes histopathological analysis of 176 specimens from 152 HS patients in order to adequately describe the disease process which occurs under the skin. Regardless of disease name, and regardless of theories of pathogenesis, this is a fresh view of the historical HS research. 2004 HS Overview Orphanet Revuz.pdf Revuz J. Hidradenitis Suppurativa <http://www.orpha.net/data/patho/GB/uk-hidradenitissuppurativa.pdf>. Accessed 4/24/2005. Orphanet, EU, 2004 March. 2004 HS Overview Orphanet Clinical sign Revuz.pdf Revuz J. Clinical signs of Hidradenitis Suppurativa- to aid diagnosis.<http://www.orpha.net/data/patho/GB/ClinicalsignsHS.pdf>. Accessed 4/24/2005. Orphanet, EU, 2004 March. Erudite and professional clinical review. 2004 Evidence review Medical Treatment of HS Jemec .pdf Jemec GB. Medical treatment of hidradenitis suppurativa. Expert Opin Pharmacother. 2004; 285(8):1767-1770. This is a plain english meta-analysis of past HS clinical studies, and an assessment of current therapies for HS. Dr Jemec suggests that theories of pathogenesis should be turned toward inflammation, and that there is an urgent need for basic research in order to effectively treat Hidradenitis Suppurativa. 2003 Treatment Variable outcomes Sartorious Et al.pdf Sartorius K, Lapins J, Emtestam L, Jemec GB. Suggestions for uniform outcome variables when reporting treatment effects in hidradenitis suppurativa. Br J Dermatol. 2003; 149(1):211-213. There have been no randomized controlled trials on surgical treatment. The authors suggest that the major barrier to making comparisons between surgical and other treatments is a lack of standardized assessment tools. Thus it is difficult, if not impossible, for doctors to review the research to determine what treatments have an adequate evidence base. The authors of this paper propose to resolve these issues by providing a standard method of patient assessment and treatment outcomes. 2003 Review HS Overview Slade Powell Mortimer.pdf Slade DE, Powell BW, Mortimer PS. Hidradenitis suppurativa: Pathogenesis and management. Br J Plast Surg. 2003; 56(5):451-461. Comprehensive review. 2003 Review Hidradenitis Suppurativa Jemec.pdf Jemec GB. Hidradenitis suppurativa. J Cutan Med Surg. 2003; 7(1):47-56. 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 3 Annotations MSAB Database Describes the confusing history of names and classification, also a good rational summary of what are the major theories of cause. 2001 HS Acne Inversa Overview Jansen.pdf Jansen T, Altmeyer P, Piewig G. Acne inversa (alias hidradenitis suppurativa). J Eur Acad Dermatol Venereol. 2001; 15(6):532-540. A slightly differing point of view from the originators of the term acne inversa, and from the authors who 'added Pilonidal sinus/cyst' to acne triad to describe the acne tetrad. 2 Prevalence_Morbidity_Epidemiology 2005 Abstract Incidence and morbidity of HS Poland Bergler-Czop.pdf Bergler-Czop B, Brzezinska-Wcislo L. (Abstract) methods for treating various forms of acne in the years 1991-2000, department of dermatology, silesian medical school. Wiad Lek. 2005; 58(3-4):156-60. During 1991 to 2000, 206 patients were hospitalized with the diagnosis of acne. Mean age of patients was 25.51 years. The following types of acne were recognized: 108 patients—acne punctata, 82--acne conglobata, 4--acne fulminant, 4-papulous-pustular acne, 8--inverse acne, respectively. 2001 Morbidity of HS Von Der Werth Jemec.pdf von der Werth JM, Jemec GB. Morbidity in patients with hidradenitis suppurativa. Br J Dermatol. 2001; 144(4):809813. Joint survey of 114 UK and Danish patients The pain, anxiety, depression and reductions in the ability to perform Activities of Daily Living of those with HS has been documented, quantified and compared to major dermatoses by these authors. 2000 Whats New In HS Jemec.pdf Jemec GB. What's new in hidradenitis suppurativa? J Eur Acad Dermatol Venereol. 2000; 14(5):340-341. 2000 Whats New In HS Jansen Plewig.pdf Jansen T, Plewig G. What's new in acne inversa (alias hidradenitis suppurativa)? Journal of the European Academy of Dermatology & Venereology. 2000; 14(5):342-343. Side by side EADV editorials discussing implications of 2000 Natural History of HS-Von Der Werth & Williams 2000 Natural History HS Von Der Werth.pdf von der Werth JM, Williams HC. The natural history of hidradenitis suppurativa. J Eur Acad Dermatol Venereol. 2000; 14(5):389-392. survey of 110 UK patients (not many males) The authors of this study have created valid epidemiological data from completed questionaires of those clinically diagnosed with HS. Characteristics such as age of onset, disease duration, familial disease, pain, number of lesions, and what aggrevates or relieves the disease are delineated. The authors make an essential point that the average duration of a single inflamed HS lesion is 6.9 days. This should be considered when deciding which treatments have an evidence base. 1999 Cigarette smoking as a trigger of HS Konig.pdf Konig A, Lehmann C, Happle R. Cigarette smoking as a triggering factor of hidradenitis suppurativa. Dermatology. 1999; 198(3):261. Investigation aim was to evaluate the smoking prevalence in HS patients. 84 patients admitted between 1990 and and 1997 for surgical treatment of moderate to severe HS in two dermatological centers (Marburg/Kassel, Germany) were asked to complete a mailed questionnaire. A control group, matched for age and sex, was randomly chosen from other skin diagnoses admissions, and a comparison to the national smoking rate was performed. Completed surveys returned by 63 persons, 36 women (57.1%) and 27 men (42.9%). Patients average age at onset of disease was 22.7 years in women and 27.4 years in men. 27% of responding patients reported at least one affected first-degree relative. 77.8% of the patients stated that their quality of life was severely impaired by the disease. ________________________________________________________________ TABLE 1 Cross-table showing proportions of active smokers and nonsmokers in 63 patients with HS, and in a matched-pair control group of patients admitted for miscellaneous other skin disease SMOKERS NONSMOKERS Cases of HS 56 (88.9%) 7 (11.1%) 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 4 Annotations MSAB Database Matched pair controls 29 (46%) 34 (54%) April 1995 National Est. 26.7% 73% _________________________________________________________________________________ The level of significance was calculated using various x2 tests (continuity, likelihood ratio, Fishers exact test). Odds ratio: 9,4: 95% confidence interval: 3.7-23.7: p<0.001 (x2 test). _______________________________________________________________________________ 56/63 active smokers (24 men, 32 women). 47.2% of these consumed more than 20 cigarettes/day 3/63 stated they were ex-smokers (4.8%) but two of these had stopped recently and after the onset of HS. 4/63 patients stated they had never smoked (5.4%). National statistics April 1995 expected smoking prevalence in Germany was 26.7% (20.4% in women and 33.4% in men). The significantly higher proportion of smokers among patients with HS can be expressed by an odds ratio of 9.4 with a 95% confidence interval of 3.7-23-7 (p<0.001). 45 smokers provided further details of smoking, 32 started smoking before the outbreak of HS (on average 10.4 Years before the first symptoms occurred. 3 subjects started to smoke in the same year as the manifestation of HS. 10 patients became active smokers after the onset of the disease (on average 5.2 years after the first symptoms ocurred). Author recommends large multi centre studies to identify the role of smoking in the pathogenesis of HS. 1996 Prevalence HS potential precursor lesions Jemec Jemec GB, Heidenheim M, Nielsen NH. The prevalence of hidradenitis suppurativa and its potential precursor lesions. J Am Acad Dermatol. 1996; 35(2 Pt 1):191-194. The purpose of this study was to determine the 1-year prevalence in a Danish population and the point prevalence of hidradenitis in consecutive patients undergoing screening for sexually transmitted diseases (STDs). Table I. Age and sex distribution of the three samples studied 1-Year Prev sample PointPrev SAMPLE Patient sample No. of male subjects 298 276 6 Mean age (yr) +- SD 42.1 +_ 14.8 28.4 _+ 8.2 37 + 11 No. of female subjects 301 231 24 Mean age _+ SD 39.3 + 14.1 25.9 _+ 7.2 35 _+ 11 Total subjects 599 507 30 Six persons (three men, three women) had a history compatible with a diagnosis of hidradenitis, which suggested a 1year prevalence of 6 of 585 (1.0%) and a 95% CI of 0.4-2.2. None of the examined persons, however, had any signs of scarring or active lesions. The point prevalence of hidradenitis in this group was 20 of 507 (4.1%)(CI = 3 - 6). The findings may also reflect an underlying heterogeneity suggestive of different subtypes of the disease. A significant pathogenetic heterogeneity of the disease would help explain the often highly variable results of medical treatment. 1996 Characteristics and consequences HS Jemec.pdf Jemec GB. Hidradenitis suppurativa - characteristics and consequences. Clin Exp Dermatol. 1996; 21(6):419. Patient characteristics and disease impact of stage 1 and stage 2 HS studied in 68 consecutive patients diagnosed with or treated for the condition during 1991-1995 via questionnaire, results cross-checked in interview. Weight and BMI assessed. 10 men(255 controls), 58 women(268 controls) various findings differed to controls- -self reported health lower in those with HS -68 HS patients lost on average 2.7 days of work in past 12 months to disease (range 0-30 days) -obesity not associated to HS in this population 1996 Case control study HS Jemec.pdf Jemec GB, Heidenheim M, Nielsen NH. A case-control study of hidradenitis suppurativa in an STD population. Acta Derm Venereol. 1996; 76(6):482-483. This study was undertaken in order to further investigate the possible relationship between hidradenitis and STD. From 1993-1995 20 diagnosed cases HS selected (I male, 19 women) 60 random case controls. Testing for syphilis and gonorrhea revealed no significant differences. Cervical smears not performed. 7/20 people with HS had condylomata, and 5/60 controls had condylomata. Chlaymdia not associated. 1989 Prevalence HS South Wales Harrison.pdf 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 5 Annotations MSAB Database Harrison B, Mudge M, Hughes L. The prevalence of hidradenitis suppurativa in South Wales. In: Marks R., Plewig G. (eds). Acne and Related Disorders. London: Martin Dunitz, 1989:365-6. Between 1978 and 1985, 150 patients with advanced HS were referred to the University Department of Surgery in Cardiff. In comparison with other reports from surgical units within the UK, the referral practice seemed considerable and led to a study of the prevalence of the disease in South Wales. METHODS-2 general practices were selected (A5652 patients) and (B-6919 patients) RESULTS: 17 women and four men identified with HS representing .25% of the 'at-risk' patient population aged 15-64 years. The observed frequencies of HS in the total practice populations were 1.4/1000 (Practice A) and 1.9/1000 (Practice B). Since 90% of the present hospital-based population experienced disease onset between the ages of 15 and 65 years, these frequencies are also expressed for the age-corrected 'at risk' practice population (15-65 years); that is 2.2/1000 (Practice A) and 2.8/1000 (Practise B). "Epidemiological studies must be the first step in realizing effective early treatment for patients with hidradenitis." 1989 Diagnostic Criteria Mortimer .pdf Mortimer P. Management of hidradenitis suppurativa. Clinical & Experimental Dermatology. 2002; 27(4):328-337. Purpose is to establish clinical criteria for the early diagnosis of HS. Information obtained from a previous study evaluating clinical markers of androgenicity in women with the disease was used to decide on the appropriate criteria. The following set of clinical criteria for early diagnosis is proposed, based on information obtained from more than 70 female patients: -Recurrent blind boils for at least 6 months in flexural sites, principally axillae and groins, that are deeply situated and 'blind'. -Onset at or after puberty, particularly during the second decade. - A poor response to conventional courses of antibiotics, with a tendency to relapse soon after or even during treatment. -The presence of comedones in apocrine gland bearing skin sites, whether clinically involved with HS or not, and the presence of retro-auricular comedones. -Routine cultures of pus from boils reveals no pathogens. -A personal family history of acne (particularly conglobate variety) and pilonidal sinus. -In women, a tendency to exacerbation of boils premenstrually. Without any biochemical or histological marker for HS, the diagnosis must depend on clearly defined clinical criteria. at present these do not exist. Development of criteria is not only important for the early recognition of the disease, but is also a prerequisite for understanding pathogenesis, and for grading disease activity. Advanced HS is a heterogenous disease, and satisfactory evaluation of treatment in controlled trials is unlikely to secceed unless groups of patients with similar disease severity and complications are compared. 3 Co-morbidity_Complications CROHN'S DISEASE (REFER ALSO TO FOLDER 6, 7 & 9.) 1997 Case report association HS epitheloid granuloma Crohn's Roy.pdf Roy MK, Appleton MA, Delicata RJ, Sharma AK, Williams GT, Carey PD. Probable association between hidradenitis suppurativa and crohn's disease: Significance of epithelioid granuloma. Br J Surg. 1997; 84(3):375-376. 48 year old man with HS histological exam of lesions showing chronic inflammatory infiltrate with multiple noncaseating granulomas of both foreign body and epitheloid type. Diagnosis of Crohn's following perianal discharge and intestinal obstructions. 1993 Comorbidity HS Crohn's Church.pdf Church JM, Fazio VW, Lavery IC, Oakley JR, Milsom JW. The differential diagnosis and comorbidity of hidradenitis suppurativa and perianal crohn's disease. Int J Colorectal Dis. 1993; 8(3):117-119. This series shows that HS is particularly likely to coexist with severe rectal or perianal Crohn's disease and that the Crohn's disease pre-dates the hidradenitis. It may be that the local swelling and inflammation associated with Crohn's disease precipitates the development of perineal HS, in patients already prone to it. This would not explain the frequent finding of axillary, groin, and buttock HS, however. Such a finding would imply a constitutional and, perhaps genetic, 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 6 Annotations MSAB Database predisposition for HS in patients with rectal Crohn's disease. The true frequency of coexistence of these 2 conditions cannot be determined from a retrospective study such as this. A prospective investigation of patients presenting with Crohn's proctocolitis is needed, where the specific possibility of HS is considered for each patient. This sort of prospective study is difficult, as any estimate of the prevalence of the two diseases depends on the ability to distinguish between them clinically. Church has been misinterpreted or very loosely interpreted by other researchers AND by HS patients. However, it is interesting to note, as Church does, that Crohn's and HS have an inherited predisposition. 1993 Granulomatous HS and cutaneous Crohn's Attanoos.pdf Attanoos RL, Appleton MAC, Hughes LE. Granulomatous hidradenitis suppurativa and cutaneous crohn's disease. Histopathology. 1993; 23(2):111. Describes three patients with HS who subsequently developed Crohn's after HS diagnosis Review clinicopathological aspects of 101 patients with HS (79 women,22 men) treated at Uni dept of of surgery between 1982 and 1992-reviewed detailed histology in 118 specimens -53 patients had axillary disease, bilateral in 29 cases, -6 females had sub-areolar disease, five had vulval skin involvement Variation in sex ratio_Axilla F:M 8:1 /Inguino-perineum F:M 2.4:1 Perianal F:M 1:1.3/Mammary skin F:M 6:0 -3/101 had co-existant Crohn's disease (2 preceding the diagnosis of HS, 1 by 8 years, 1 by 10 years, no info on third). 2/101 females had ulcerative colitis (preceding HS diagnosis) 1/101 had cutaneous lupus (HS preceding Crohn's Dx by three years) 1/101 had pulmonary sarcoidosis 19/101 had acne vulgaris 2/101 developed invasive SCC within chronic HS, 1 anal, 1 vulval 118 SKIN RESECTIONS -keratotic follicular plugging the only consistent feature -51 cases stratified squamous epithelial lined sinus tracts (71% of perianal resections/28% of axillary resections/43% perineal resections). -Adnexal inflammation primarily related to hair follicle -Perifollicultis in 78 (66%) -active apocrine inflammation in 14 (12%) -active eccrine inflammation in 30 (25%) -Foreign body type granuloma in 29 resections commonly adjacent to sinus tracts, ruptured epidermal cysts or around inflamed hair follicles. -Discrete epitheloid granulomas in the deep dermis and subcutis, away from the inflamed tissues or sinus tracks were uncommonly identified in only 10 resections Discrete epitheloid granulomas away from any inflammatory focus have not been described in HS but were present in all three original cases with Crohn's and in 10 of our 118 (8%) skin resections....this should not necessarily be considered to represent crohn's disease or sarcoidosis as morphologically indistinguishable granulomas occur in the abscence of systemic disease....the definitive diagnosis of perineal/perianl HS in a patient with intestinal Crohn's disease may not be possible. However, cutaneous disease extending up the anal canal would exclude the diagnosis of HS alone. In axillary cases, with the correct clinical picture, differentiation of HS from cutaneous Crohn's should be easier. 1992 HS and Crohn's in families Gower_Rousseau.pdf Gower-Rousseau C, Maunoury V, Colombel JF, et al. Hidradenitis suppurativa and crohn's disease in two families: A significant association? Am J Gastroenterol. 1992; 87(7):928. Cases of co-existence of HS and Crohns in two familes, not evidence of familial transmission but proposes there may be a similar genetic predisposition. 1991 HS in Crohn's Disease Ostlere.pdf Ostlere LS, Langtry JA, Mortimer PS, Staughton RC. Hidradenitis suppurativa in crohn's disease. Br J Dermatol. 1991; 125(4):384-386. Three patients with Crohn's disease who developed the clinical features of hidradenitis suppurativa (HS) are reported. To our knowledge this association has not been previously described. These cases illustrate the similarity between cutaneous Crohn's and ano-genital HS once chronic and established. CARCINOMA 2005 HS Squamous Cell Carcinoma Paraneoplastic neuropathy Rosenzweig.pdf 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 7 Annotations MSAB Database Rosenzweig LB, Brett AS, Lefaivre JF, Vandersteenhoven JJ. Hidradenitis suppurativa complicated by squamous cell carcinoma and paraneoplastic neuropathy. Am J Med Sci. 2005; 329(3):150-152. 50 year old man 20 year history of extensive perineal HS hospitalized due to several months of progressive lower extremity weakness. Exams unremarkable, surgical excisions (*3) showed extensive, SCC. Following third excision of wide margins for SCC, patients neurological symptoms improved, at 18 months patient fully functional. 2005 HS Vulval SCC Short.pdf Short K, Kalu G, Mortimer P, Higgins E. Vulval squamous cell carcinoma arising in chronic hidradenitis suppurativa. Clinical & Experimental Dermatology. 2005 June 23; Epub ahead of print. 57 year old woman 20 year hx of Crohn's disease, presents with white hyperkeratic nodule with a central crater or fissure at the superior aspect of the right labium minora which was clinically suspicious of a SCC. Recent immunosuppressive therapy for nephrotic syndrome secondary to membranous glomerulonephritis. Diagnosis of HS given 15 years ago, for perineal and genital areas. Lesion excised, SCC confirmed, also features of HS "We suggest constant vigilance with regard to malignant change in ano-genital HS as the diagnosis can be difficult. A painful lump or ulcer could easily be mistaken for an inflammatory lesion such as an abcess or sinus and a low threshold for biopsy is warranted. In one reprted case, seven negative biopsies were obtained before a histological diagnosis of SCC was made emphasizing the need for repeated, deep biopsy in any atypical lesion ocurring in this condition." 2001 Cancer Incidence HS LAPINS.pdf Lapins J, Ye W, Nyren O, Emtestam L. Incidence of cancer among patients with hidradenitis suppurativa. Arch Dermatol. 2001; 137(6):730-734. 2119 patients included in national cohort study. Essential consideration for physicians: "Clinicians caring for patients with HS should be aware of their propensity to develop these tumours. Whenever atypical clinical manifestations of HS occur, histopathologic examination of representative skin biopsy specimens are recommended." 2000 Verrucous Carcinoma arising in HS Cosman.pdf Cosman BC, OGrady TC, Pekarske S. Verrucous carcinoma arising in hidradenitis suppurativa. Int J Colorectal Dis. 2000; 15(5/6):342-346. 47 year old man 10-20 year history of HS, never sought treatment prior to presentation for bilateral chronically inflamed inner buttocks, excision revealed verrucous carcinoma left buttock, features consistent with HPV however PCR for HPV negative Reviews 47 cases of SCC complicating HS, reviews 46 cases of SCC complicating Pilonidal sinus, reviews 1 case of SCC complicating Dissecting cellulitis of scalp, reviews 1 case of SCC complicating Acne Conglobata . Pilonidal sinus and HS are still widely considered separate diseases, and no one has as yet made the leap to say that all pilonidal sinuses are manifestations of acne inversa. More inexplicably, acne conglobata and HS persist in the literature as separate entities. Clearly there is a need for consistent, agreed-upon terminology that takes into account location, gross appearance, and histologic characteristics of this family of lesions. of HS and its related lesions occurs with a frequency that is unquantified, but enough to generate almost 100 case reports. In Jackman’s review of 125 consecutive cases of perianal HS, 4 (3%) had squamous cancer [32]. The actual incidence is probably lower than this. Whatever the incidence of malignant change, we recommend early surgical excision of established HS lesions, especially in the anogenital region. “Conservative” measures such as avoidance of tight clothing and maintenance of good hygiene hark back to obsolete theories of the etiology of HS and are of no known use. Antibiotics work only for acute infection, a secondary problem, and the granular tracts remain to be addressed. Retinoids may limit the formation of new lesions and are a reasonable adjunct to resection, both to avoid “recurrence” at the edges of the resection and to treat the rest of the patient. However, there has been mixed success in treating established lesions with isotretinoin. Excision is safe and effects a local cure if all tracts are followed and all chronically inflamed skin is excised. Even large excisions can be left to granulate. 1999 Vulval SCC arising in chronic HS Manolitsos.pdf Manolitsas T, Biankin S, Jaworski R, Wain G. Vulval squamous cell carcinoma arising in chronic hidradenitis suppurativa. Gynecol Oncol. 1999; 75(2):285-288. 52 y.o. Maltese/Australian female, 30 year history HS, patients father and2/7 siblings also diagnosed with HS. Right vulval mass for 8 years then excised, histology reports non-malignant HS. Lesion recurred soon after excission, increasing in size, presents for treatment two years later. Lesion biopsied, could not distinguish between 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 8 Annotations MSAB Database pseudoepithiomatous hyperplasia and well-differnetiated SCC. Histology following excision showed well-differntiated SCC. Uncomplicated recovery. Malignancy should always be considered in the prescence of a vulval mass and adequate biopsies taken. 1996 SCC In Verneuils disease Malaguarnera.pdf Malaguarnera M. Squamous-cell cancer in verneuil's disease (hidradenitis suppurativa). The Lancet. 1996; 348(9039):1449. 66 y.o. male presents with painful swelling in the perineal region. 20 year history of HS treated with antibiotics and drainage, resolving spontaneously. Lab tests-fraction C3, C4 complement deficiency, inverted CD4+,CD8+ ratio. CT Scan showed solid hypodense tissue between the intergluteal fold and rectal area, extending to the last sacral vertebrae. Treatment: Nodule drained, SA and Strep Milleri and SCC found radical excision, primary closure, metastes in inguinal lymph nodes, liver metastases found on CT scan Patient died 7 months after diagnosis. 1996 HS bilateral vulvar masses Goldberg.pdf Goldberg JM, Buchler DA, Dibbell DG. Advanced hidradenitis suppurativa presenting with bilateral vulvar masses. Gynecol Oncol. 1996; 60(3):494-497. 41 yo female presents with non-malignant bilateral vulval masses (20cm*10cm), axillary boils and draining sinus tracts ten years prior to presentation. Patient sought no medical treatment despite vulval symptoms lasting ten years. Skin changes, scarring and sinus tracts from the pubis, across the inguinal region and to the iliac crest on the buttocks. Vulval disease diagnosed as HS. Treated with antibiotics oral and topical for four weeks prior to surgical removal. Condition recurred 2 months after surgery, no recurrence after 48 months. OTHER 2006 Surgical morbidity GMCSF for Perianal HS Sharon Guidetti.pdf Sharon-Guidetti, A.;Ziv, Y.;Kummer, E.;Yogev, R.;Halevy, A. Granulocyte-Macrophage Colony-Stimulating Factor for Perianal Hidradenitis Suppurativa: Report of a Case. Dis Colon Rectum. 2006 Jan 31; [Epub ahead of print] MORBIDITY and COMPLICATIONS OF SURGERY FOR HS, Novel wound healing treatment ...We describe the first patient, to the best of our knowledge, with long-standing, unresolved PHS in whom repeated perilesional injections of granulocyte macrophage colony-stimulating factor (rh GM-CSF, Molgramostim, Novartis) in addition to the surgical procedures, resulted in an uneventful recovery.... Before admission he had been operated on 16 times in another country, with repeated incision and drainage of abscesses and wide excisions of multiple sinuses. Six weeks later, wide excisions of multiple sinuses and fistulas were performed leaving open wounds for secondary healing. During the follow-up period, recurrent abscesses were drained and repeated excisions were performed. An ischiorectal abscess spontaneously drained into the anal canal at a distance of 5 cm from the anal verge, and outside into the perineum, 3 cm from the anal orifice, followed by the creation of a high, transsphincteric fistula (TSF). The TSF was treated by the insertion of a draining seton, followed by the creation of a diverting colostomy. The patient still needed reexcisions of multiple sinuses, and repeated fistulotomies were performed. Treatment with hyperbaric oxygen was unsuccessful. We performed a rectal advanced flap, after removing the seton, and the TSF healed. Because of continuous unresolved flare-ups of PHS, we decided to add GM-CSF perilesional injections to the wide excision surgical procedures. Rapid wound healing of multiple lesions was achieved with normal-looking scars. The colostomy was closed six months after the first GM-CSF injection, and two years later the patient is in good condition with no symptoms of PHS... In the last decade, experience with the use of GMCSF and its effect on wound healing has been accumulated. GM-CSF affects the cells that participate in the process of wound healing, such as neutrophils, monocytes, macrophages, lymphocytes, and endothelial cells.6,7 GM-CSF plays a major role in Langerhans cell maturation, keratinocyte growth, and fibroblastic proliferation, promoting rapid wound healing.8 Treatment of chronic wounds, such as leg ulcers of various etiologies, with GM-CSF, has shown good results in accelerating wound healing,9 making GM-CSF the treatment of choice for complicated wound healing, as in the case of our patient. Adding GM-CSF in cases of severe, long-standing, unresolved PHS should be considered among the various treatments. 2005 PG associated with HS Ah-Weng.pdf 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 9 Annotations MSAB Database Ah-Weng A, Langtry J, Velangi S, Evans C, Douglas W. Pyoderma gangrenosum associated with hidradenitis suppurativa. Clin Exp Dermatol. 2005 Nov; 30(6):669-71. 6 patients with HS (3 women, 3 men) who later developed co-existant and refractory Pyoderma Gangrenosum, including one case of superficial PG superimposed over axillary HS in one male (photo). Includes review of past cases 2004 HS Massive enlargement of Penis Baughman.pdf Baughman SM, Cespedes RD. Unusual presentation of hidradenitis suppurativa with massive enlargement of penis. Urology. 2004; 64(2):377-378. Our patient was a 55-year-old black man with active HS for nearly two decades. Despite multiple surgical procedures for severe inguinal, gluteal, and perianal disease, extensive disease persisted. Ten years of persistent, extensive penile swelling warranted urologic referral. On examination, he presented with a markedly enlarged, indurated penis, measuring 15 20 cm (Fig. 1). The ventral penis was massively enlarged, resulting in an inferior displacement of otherwise normal lower abdominal skin to the dorsal penis. Despite inflammatory and fibrotic changes at the gluteal cleft and perineal tissues, the scrotum was uninvolved. No fistulas, malignancy, or urethral abnormalities were noted on examination or radiologic evaluation. Post-surgical follow-up- Minimal recurrent induration, normal cutaneous sensation, and normal voiding and erectile function were noted at 3 years of followup. Discussion: Although HS affecting the penis has not been reported, successful resolution of HS with scrotal elephantiasis,1 urethral fistula,2 and partial scrotal excision with grafting3 have been reported. Most cases appear to involve the thinner scrotal skin, which is more susceptible to the edematous changes from chronic inguinal inflammation. An STSG would appear to be the treatment of choice in most circumstances; however, STSGs are insensate, can shrink up to 50%, and can potentially leave an unsightly mesh appearance—undesirable characteristics for penile reconstruction. Additionally, active HS involvement in close proximity risks graft infection and loss. A local vascularized tissue transfer was not possible, because all available skin was scarred from previous HS infections. Therefore, the translocated abdominal skin provided the necessary “skin graft” and continued to perform well at last follow-up. 2003 Sclerotic changes due to Isotretinoin Rx for HS Atalay.pdf Atalay, A.;Altaykan, A.;Ergin, G.;Kutsal, YG. Reversible sclerotic changes of lumbar spine and femur due to longterm oral isotretinoin therapy. Rheumatol Int. 2004 Sep;24(5):297-300. Epub 2003 Sep 10. MORBIDITY AND COMPLICATION OF MEDICAL TREATMENT FOR HS-ISOTRETINOIN INDUCED BONE CHANGES AND SPONDYLOARTHROPATHY The patient fulfilled the clinical diagnostic criteria of Follicular Occlusion Triad: dissecting cellulites, hidradenitis suppurativa and acne conglobata. 2002 Twenty five HS patients Laparoscopic Illeostomy Swain.pdf Swain BT, Ellis CNJ. Laparoscopy-assisted loop ileostomy: An acceptable option for temporary fecaldiversion after anorectal surgery. Dis Colon Rectum. 2002 May; 45(5):705-7. “A retrospective review of the medical records of 53 consecutive patients who underwent laparoscopy-assisted creation of a loop ileostomy for temporary fecal diversion as an adjunct to anorectal or perineal surgery between 1993 and 1999 was performed... The anorectal or perineal surgical procedures included excision of perianal hidradenitis suppurativa (25 patients)” Therefore the frequency of HS in those presenting for “laparoscopy-assisted creation of a loop ileostomy for temporary fecal diversion as an adjunct to anorectal or perineal surgery” is 47%. 1999 Severe HS contributing to death and review Groginsky.pdf Groginsky EM, Olson JK, Lallas TA, Buekers TE, Potts S, Sorosky JI. A case of severe hidradenitis suppurativa contributing to a death and a review of the literature. Journal of Lower Genital Tract Disease. 1999; 3(1):67-70. HS was was not the primary condition which led to death in this case. However untreated HS over a 20 year period was A MAJOR CONTRIBUTING FACTOR leading to this ladys death. LIST OF FINDINGS IN THIS LADYS CONDITION: Fatigue, Anoroxia,30 pound weight loss over 6 months, Difficulty in urinating, Diarrhoea for 2 months, 20 year history of vulvar and axillary HS, Pitting lymphedema from ankles to groin, Skin over pubic and perineal area replaced by sinus tracts and fistulas, Pubic bone visible through fistulas, Sinus tracts extending from perineum over buttocks to the hips, Free air under diaphragm (from fistulas and sinus tracts), Liver enlargement, Confirmed on 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 10 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database autopsy as NOT having Crohn's disease, Sepsis, Sigmoid Colon Diverticulitis, Perforated Sigmoid Diverticulum, Peritonitis with fibrous growths and pus covering the stomach wall and covering the internal organs, Vulvar lesion the approximate size and volume of three packets of cigarettes stuck together, Vulvar lesion covering openings to urethra and anus, Right Ovarian Seroma Cystadenoma, Perforated Sigmoid Colon, Multi-System Failure. 1997 ABSTRACT HS inducing secondary systemic amyloidosis Black.pdf Black RJ, Scott KW, Allen DE. Hidradenitis suppurativa inducing secondary systemic amyloidosis - A case report. Journal of the European Academy of Dermatology and Venereology. 1997; 9(1001):126-126. 59 year old female, 19 year history of HS. Age 53 diagnosed with SSA by skin and gut biopsy during admission for malabsorption diarrhoea and HS flare. GI and renal tract amyloidosis slowly developed prior to patient death. 1968 Anemia HS Tennant.pdf Tennant F,Jr, Bergeron JR, Stone OJ, Mullins JF. Anemia associated with hidradenitis suppurativa. Arch Dermatol. 1968; 98(2):138-140. In 42 patients with HS surveyed, ten associated instances of marked anemia were found. All ten patients had severe HS for a minimum of 2 years. The 42 cases of HS reviewed were consecutive and not selected for duration. Further studies on the relationship between HS and anemia are indicated. (42 of 54 patients with HS admitted to the University of Texas medical Branch between 1952 and 1967 were selected for the study.) Ages of the patients ranged from 18-66 years and there were 22 men and 20 women. HS should be added to the list of chronic infectious diseases that are associated with anemia. The etiologic role of the organisms in HS has never been clearly defined. Whether the organisms cause HS or whether they are secondary invaders, they nonetheless exert a systemic toxic effect on the patient. Exact mechanisms by which anemia is produced in HS is unknown. 1967 Interstitial keratitis HS Bergeron.pdf Bergeron JR, Stone OJ. Interstitial keratitis associated with hidradenitis suppurativa. Arch Dermatol. 1967; 95(5):473475. In 62 consecutive patients with HS who were surveyed, four associated cases of interstitial keratitis was found (3 black men, 1 black woman all with HS in mutiple sites, and their condition ranged from moderately severe to severe). 3/4 had negative serologic tests for syphilis and nonreactive Treponema pallidum immobilization or flourescent treponemal antibody absorption (FTA-ABS) tests. The syphilis in the fourth patient was of the acquired type. None of the four patients with interstitial keratis had any of the stigmata of congenital syphilis. We believe that there should be further studies on the relationship between HS, a chronic granulomatous disease and interstitial keratitis. 1966 HS complications death Moschella.pdf Moschella SL. Hidradenitis suppurativa. complications resulting in death. JAMA. 1966; 198(1):201-203. 37 year old black man had an extensive follicular occlusion triad, with severe perianl and perineal HS. Had rare complications of fistulas to the rectum, urethra, urinary bladder, and peritoneum and severe hypochromic anemia, hypoproteinemia and amyloidosis. His death as a result of HS is very unusual. "The disease occurs equally in both sexes at or after puberty". 4 Pathogenesis_Cellular_Molecular_Genetic 1957 Experimental production of Acne Shelley Kligman.pdf Shelley W, Kligman A . The experimental production of acne by penta and hexachloronapthalenes. Arch Dermatol. 1957 May; 75:689-694. Reported induction of HS-like and acne-conglobata disease by the dermal application of halowax 1014. Subjects were volunteers from Holmesburg prison PA, United States. 1955 Experimental HS pathogenesis Shelley.pdf Shelley W, Cahn M. The pathogenesis of hidradenitis suppurativa in man; experimental and histologic observations. AMA Arch Derm. 1955; 72(6):562-565. Reported induction of apocrine HS. 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 11 Annotations MSAB Database HISTOPATHOLOGY BACTERIOLOGY IMAGING 2006 Cathepsin L hurpin Acne Inversa Bylaite.pdf Bylaite, M.;Moussali, H.;Marciukaitiene, I.;Ruzicka, T.;Walz, M. Expression of cathepsin L and its inhibitor hurpin in inflammatory and neoplastic skin diseases. Exp Dermatol. 2006 Feb;15(2):110-8. CELLULAR EVIDENCE OF INFLAMMATORY PROTEINS IN ACNE INVERSA Recently, cathepsins (Cats), belonging to the lysosomal cysteine proteinases, were particularly emphasized for their activity in important biological functions, such as proteolytic processing of proenzymes, antigen presentation, inflammation, hormone maturation, fertilization, tissue remodelling, bone matrix resorption, cell proliferation, differentiation, apoptosis and degradation of the extracellular matrix, facilitating wound healing but also the growth and invasion of tumor cells Over the last decade, Cat L has been of great interest due to its numerous functions. Cat L appeared to be critically involved in epidermal homeostasis, regulation of the hair cycle and MHC class II-mediated antigen presentation in cortical epithelial cells of the thymus (10,11). The expression of Cat L in the thymus is essential for natural killer T (NKT) cell development (12). Surprisingly, Cat L-deficient mice were shown to develop epidermal hyperproliferation and periodic hair loss (7). In previous work, it has been shown that Cat L is inhibited by hurpin/headpin (22–24). Hurpin, a serine proteinase inhibitor, was originally discovered as an UV-repressible gene in HaCaT cells (24). It was independently described as headpin by others and shown to be differentially expressed in the head and neck cancer (25). Hurpin is closely related (59% amino acid identity) to the squamous cell carcinoma antigen (SCCA) 1 and 2 (24)..Previously, we have shown that the expression of hurpin is associated with the activation or proliferation state of keratinocytes (24). Interestingly, hurpin was found to be overexpressed in psoriatic skin lesions compared with normal skin (24). On the basis of the observation that hurpin protects keratinocytes from UV-induced apoptosis, it was linked to differentiation and apoptosis of human keratinocytes....Additionally, we analysed Cat L and hurpin expression in bacterial and secondary infected diseases, such as infectious folliculitis (Fig. 1i,j), acne inversa, pyoderma gangrenosum and chronic leg ulcers. The epidermal localization and intensity of Cat L and hurpin was similar to that seen in chronic inflammatory dermatoses..... The strongest expression of Cat L was observed in the spinous layer of psoriasis, atopic eczema and SCC and in the basal layer in samples of acne inversa. 2005 Sirolimus adverse effects-HS Mahe.pdf Mahe E, Morelon E, Lechaton S, et al. Cutaneous adverse events in renal transplant recipients receiving sirolimusbased therapy. Transplantation. 2005; 79(4):476-482. Possible trigger for HS. Sirolimus administered to renal transplant patients. Ten patients (12%) demonstrated hidradenitis suppurativa located in axillary, perianal, and inguinal areas. This was neither sex-dependent nor combined with acne-like eruptions. 2004 Keratinocytes hair follicles sinus tracts of HS Gniadecki Jemec.pdf Gniadecki R, Jemec GBE. Lipid raft-enriched stem cell-like keratinocytes in the epidermis, hair follicles and sinus tracts in hidradenitis suppurativa. Exp Dermatol. 2004; 13(6):361-363. Pathogenesis/ Demonstration of lipid rafts and cell types found in HS lesional/paralesional skin samples via confocal laser-scanning fluorescence microscope An important feature of HS is the formation of sinus tracts in the dermis and subcutis, which is thought to occur secondary to follicular inflammation and/or rupture and infiltrative growth of follicle cells in the dermis and subcutis (3). Histologically, the keratinocytes of the sinus tracts appear to grow into the surrounding tissues without any absence of compression of adjacent structures. Development of sinus tracts is an important pathogenic step, as it is a hallmark of a chronic recurrent course of the disease. ..sections of the skin obtained from the lesional and paralesional skins from five patients with HS undergoing routine surgical treatment (2 axillary/3 genitofemoral lesions, m/f: 1/4, age 18–36 years, duration of disease 2–16 years) It has long been known that a mechanical damage of the deeper parts of the hair follicles in the Rhino mouse causes sinus tract formation (11). We wish to propose that, in HS, the CD29brightCTxbright cells derived from the epidermis and/or hair follicles migrate from the sites of epithelial damage into the dermis forming sinus tracts. In some sections of the lesional HS skin we found epidermal CD29brightCTxbright cells in a continuum with the underlying sinus tracts (Fig. 1e), an observation that supports this hypothesis. Furthermore, transgenic mice overexpressing b1 integrin (CD29) in the epidermis develop a psoriasiform phenotype characterized by inflammation, epidermal hyperproliferation, and perturbed differentiation (12,13). The CD29bright CTxbright phenotype may be a marker of activated cells, migratory cells, like those seen in cultured keratinocytes or in vivo during wound healing (10). Thus, sinus tract formation may represent an aberrant 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 12 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database epidermal repair response executed by the activated CD29bright CTxbright keratinocytes capable of non-malignant infiltrative growth in the dermis and subcutis. 2004 Lithium hidradenitis Aithal IJDLV.pdf Case study of HS developing 9 years after commencing Lithium treatment. Our patient finally improved only after lithium was tapered and stopped although controllable recurrences followed. HS due to lithium could possibly be caused due to neutrophilic chemotaxis and their degranulation inducing the inflammatory cascade (as in psoriasis). Follicular plugging due to direct influence of lithium on the follicular keratinocytes (as in acne) resulting in follicular occlusion adds to the pathology. (Lithium administration may also lead to TNFa toxicity) 2002 Immunohistochemical eval vulvar HS Heller.pdf Heller DS, Haefner HK, Hameed M, Lieberman RW. Vulvar hidradenitis suppurativa. immunohistochemical evaluation of apocrine and eccrine involvement. J Reprod Med. 2002; 47(9):695- 700. Histopathology of 13 vulvar case HS plus comparison to two female axillas and one male axilla Confirms a predominance of eccrine over apocrine glands in vulvar region, involvement of both types of glands in Hs is a secondary event. Majority of eccrine glands in active areas. Apocrine glands when present were away from active inflammation. Inflammation and eventual destruction of the glands appears to be part of a secondary process. 2001 Neutrophil-related Host response Inactive HS Lapins et al.pdf Lapins J, Asman B, Gustafsson A, Bergstrom K, Emtestam L. Neutrophil-related host response in hidradenitis suppurativa: A pilot study in patients with inactive disease. Acta Dermatovenereologica. 2001; 81(2):96-99. The purpose of this pilot study was to compare the release of oxygen radicals and primary granular release from in vitro activated peripheral neutrophils from HS patients with inactive disease and healthy controls. A further objective was to describe the degree of activation measured as receptor expression. The patients with HS consisted of 15 women (mean age 46 years, range 27–57) and the control group of 15 age-matched healthy women (mean age 46 years, range 25–56). Acute phase reactants and blood cell counts all within normal ranges and comparable to controls (except higher haptoglobin mean concentration compared to controls yet within normal range also). The generation of free oxygen radicals was significantly higher. Therefore, it is suggested that a defect in the function of neutrophils might be of pathogeneic importance in HS, but further functional studies are needed to elucidate the merits of the case. 1999 Coagulase Negative Staph CO2 laser surgery cultures Lapins.pdf Lapins J, Jarstrand C, Emtestam L. Coagulase-negative staphylococci are the most common bacteria found in cultures from the deep portions of hidradenitis suppurativa lesions, as obtained by carbon dioxide laser surgery. Br J Dermatol. 1999; 140(1):90-95. In this study, 22 women and three men with a mean age of 35·3 years and a mean HS duration of 10·6 years were treated with this CO2 laser surgical method. Our study was undertaken to characterize a representative bacterial milieu in the deeper parts of HS. In an attempt to get a sufficient bacterial yield for culture and to minimize interference from resident skin bacteria, we used a technique for the treatment of HS aiming at complete carbon dioxide (CO2) laser ablation of diseased tissue and preservation of healthy tissue.8 Twenty-five patients, 22 women and three men, mean age 35·3 years (range 19–49), were studied. All patients were culture-positive from at least some level Staphylococcus aureus and coagulase-negative staphylococci (CNS) were the species most frequently found After the staphylococci the anaerobic Peptostreptococcus species and Propionibacterium acnes were most common, In conclusion, our results indicate that bacterial infection is common and sterile inflammatory lesions are exceptional in HS. The infections with aerobic and/ or anaerobic bacteria are probably secondary in a structurally abnormal tissue. The frequent findings of CNS in the deeper parts of HS suggest that they have a pathogenic significance. In future studies, speciation of the CNS might shed more light on their pathogenic potential. The presumed destructive properties of these otherwise harmless members of the skin commensal flora could be secondary to mechanisms similar to the ones involved in the foreign body situation. 1997 ABSTRACT Dilated pores Epidermal Metaplasia HS Dammert.pdf Dammert KF. True dilated pores in skin subject to chronic hidradenitis suppurativa: Epidermal metaplasia of infundibula. The American Journal of Dermatopathology. 1997 Oct; Volume 19(5):513. 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 13 Annotations MSAB Database I case study 1 person with HS found to have half of apocrine follicles dilated, diagnosed as epidermal metaplasia of infundibula, have not seen this cited elsewhere. 1997 Histology HS lesions and intra individual variation Jemec.pdf Jemec GB, Thomsen BM, Hansen U. The homogeneity of hidradenitis suppurativa lesions. A histological study of intra-individual variation. APMIS. 1997; 105(5):378-383. A total of 51 specimens were taken from 11 patients (2 men, 9 women; 33-48 years; mean age 41 years, 95% confidence interval). Seven patients had lesions excised from two or more different anatomical regions, i.e., groin and axilla or breast and axilla. Overall, 30 specimens were taken as synchronous biopsies, while the remainder were taken over a period of time ranging from 2 months to 6 years. Six patients had both synchronous and consecutive specimens. The histological features are shown in chronological order, where appropriate, in Table 1. Sinus tracts were found in 33/51 specimens (Fig. 1), paralesional evidence of poral occlusion in 25/51 (Fig. 2), abscesses in 12/51 diffuse dermal inflammation in 10/51 (Fig. 3) folliculitis without poral occlusion was seen in 9/51, as were cysts (9/51), and primary eccrine gland involvement in 1/51. Primary apocrine gland involvement was not observed. Poral occlusion, sinus tract formation and cysts were found in 44/51 specimens, and in all 17 specimens from 5 patients. Only seven specimens differed from this pattern. three specimens showed acute inflammation of unknown origin, three specimens showed extensive fibrosis and chronic inflammation, and one specimen showed areas with features of both. All specimens presented a number of minor features (Table 2): dermal fibrosis was seen in 33/51, free hairs in 18/51, secondary eccrine involvement in 10/51(Fig. 4), secondary apocrine involvement in 7/51 (Fig. 5), and granulation tissue formation in 3/51. Plotting the number of specimens with fibrosis and diffuse dermal inflammation against the total number of specimens from the patients revealed a significant positive linear correlation (R2=0.53, p=0.011). The qualitative assessment of the generally mixed inflammatory infiltrate revealed that +++ infiltrates of lymphocytes were present in 38/51, neutrophils in 29/51, histiocystic cells in 24/51, giant cells in 20/51, eosinophils in 3/51, and plasma cells in 2/51. This study confirms that the histological pattern of hidradenitis, irrespective of topographical location or overall duration of the disease, is that of a follicular disease. The simultaneous occurrence of these changes in distant regions (e.g. axilla and groin), as well as over time, also suggests that generalized individual anatomical abnormalities are involved. The aetiology,meanwhile, remains unknown. 1997 Ultrasound hair follicles HS Jemec.pdf Jemec GB, Gniadecka M. Ultrasound examination of hair follicles in hidradenitis suppurativa. Arch Dermatol. 1997; 133(8):967-970. We undertook this study to investigate the qualitative and quantitative aspects of in vivo structure of hidradenitis suppurativa visualized using ultrasound. patients with hidradenitis suppurativa (n=15; 1 man and 14 women; mean +/SD age, 35.8 +/- 11.3 years) and healthy control subjects (n=13; 1 man and 12 women; mean +/- SD age, 32.4 +/- 9.7 years). Paralesional clinically uninvolved hair follicles of patients with hidradenitis had a greater diameter and a distorted shape with a wider deep follicle diameter, while the superficial diameter appeared normal. This caused a characteristic echographic profile resembling that of an arrow pointing toward the entrance echo (Figure 2). This was found in the majority of the clinically uninvolved hair follicles of the axilla and the genitofemoral area. In contrast to hair follicles in controls, it was generally easy to measure the diameter of hair follicles in patients with hidradenitis. Overall skin thickness, follicle area, and hair follicle shape were accessible for quantitative analysis (Table 1). Comparison of clinically uninvolved skin showed an increased axillary skin thickness P =.009) and axillary follicle area (P=.002) in patients with hidradenitis, whereas the axillary follicle shape was not significantly different from that of controls. In the genitofemoral area, a greater skin thickness (P=.005) and significantly different follicle shape (P=.007) were seen, without significant differences in follicle area. Ultrasound examination, therefore, may be of practical use in the preoperative assessment of hidradenitis lesions to minimize the size of excisions. 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 14 Annotations MSAB Database 1996 HS OR AI clinicopathological study early lesions Boer.pdf Boer J, Weltevreden EF. Hidradenitis suppurativa or acne inversa. A clinicopathological study of early lesions. Br J Dermatol. 1996; 135(5):721-725. 27 consecutive patients, 26 females and 1 male, clinical and hstopathological data investigating histopathology of early lesions (disease activity, extent,severity and duration of biopsied lesions). Results confirm that stasis and destruction of apocrine glands appear to be secondary phenomena Results suggest immune mediated response, discuss similarities to acne-High percentage of HLA-DR lymphocytes/ Lue-8-positve immunoregulatory lymphocytes present in lower numbers Refer table histopathological diagnostic clues for HS1 perifollicultis 2 spongiosis, infundibulofolliculitis 3 occlusion 4 dilatation follicle 5 stasis of apocrine and/or eccrine glands 6 rupture of follicle 7 inflammation of the glands 8 abcess or granuloma formation 9 panniculitis 10 fibrosis or sinus 1996 Bacteriology of HS Jemec.pdf Jemec GB, Faber M, Gutschik E, Wendelboe P. The bacteriology of hidradenitis suppurativa. Dermatology. 1996; 193(3):203-206. Study undertaken in order to investigate the bacteriology of early lesions of HS with the aim of identifying the bacterial flora and the antibody response to streptococci and staphylococci. Microbiological samples-taken by aspirating lesion. Bacteria typed and blood sample for detection of IgG-precipitating antibodies to bacterial antigens was taken. 41 patients examined: 21/41 aspirates sterile 51% S Aureus cultured in 8/41 S epidermis cultured in 11/41 S hominis S Milleri cultured in 1/41 Polymicrobial cultured 4/41 A subgroup of 32 patients had blood samples drawn for studies of precipitins. The number of S aureus precipitates approached significance but no specific response was seen in patients in whom S Aureus was detected. 1995 Pathogenesis of HS Attanoos.pdf Attanoos RL, Appleton MA, Douglas-Jones AG. The pathogenesis of hidradenitis suppurativa: A closer look at apocrine and apoeccrine glands. Br J Dermatol. 1995; 133(2):254-258. Review clinicopathological aspects of 101 patients with HS (79 women,22 men) treated at Uni dept of of surgery between 1982 and 1992-reviewed detailed histology in 118 specimens Control group-four women and six men -53 patients had axillary disease, bilateral in 29 cases, -6 females had sub-areolar disease, five had vulval skin involvement Variation in sex ratio_Axilla F:M 8:1 /Inguino-perineum F:M 2.4:1 Perianal F:M 1:1.3/Mammary skin F:M 6:0 -3/101 had co-existant Crohn's disease (2 preceding the diagnosis of HS, 1 by 8 years, 1 by 10 years, no info on third). 2/101 females had ulcerative colitis (preceding HS diagnosis) 1/101 had cutaneous lupus (HS preceding Crohn's Dx by three years) 1/101 had pulmonary sarcoidosis 19/101 had acne vulgaris 2/101 developed invasive SCC within chronic HS, 1 anal, 1 vulval 118 SKIN RESECTIONS -keratotic follicular plugging the only consistent feature -51 cases stratified squamous epithelial lined sinus tracts (71% of perianal resections/28% of axillary resections/43% perineal resections). -Adnexal inflammation primarily related to hair follicle -Perifollicultis in 78 (66%) -active apocrine inflammation in 14 (12%) -active eccrine inflammation in 30 (25%) 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 15 Annotations MSAB Database The histopathological findings in HS appear to be diverse and non-specific. The only consistent feature was marked follicular plugging. The universal finding of such a feature, particularly in view of its abscence from the control series, suggests that it is an early and essential element in the pathogenesis of HS. 1995 MMP Exocrine epithelium high expression axillary HS SaarialhoKere.pdf Saarialho-Kere U.K., Crouch EC, Parks WC. Matrix metalloproteinase matrilysin is constitutively expressed in adult human exocrine epithelium;extracts. J Invest Dermatol. 1995; 105(2):190-196. Matrix metalloproteinase matrilysin In axillary hidradenitis , prominent immunostaining was seen in the epithelium of apocrine glands (Fig 2E) similar to that in normal apocrine glands (data not shown). Figure 2E E In axillary hidradenitis, strong signal for matrilysin protein was detected in all duct epithelial cells. 1995 10 HS biopsies TNFa immunoreactivity apocrine Ahmed.pdf Ahmed AA;Nordlind K;Schultzberg M;Lidén S Immunohistochemical localization of IL-1 alpha-, IL-1 beta-, IL-6- and TNF-alpha-like immunoreactivities in human apocrine glands. Arch Dermatol Res 1995;287(8):764-6 Aim: Immunoreactivities to IL-1 alpha-, IL-1 beta-, IL-6- and TNF-alpha have been found in eccrine sweat gland apparutus, and IL-1 has been found in eccrine sweat. However immunoreactivities for these cytokines in apocrine glands have so far not been reported. We report here the distribution of IL-1 alpha-, IL-1 beta-, IL-6- and TNF-alpha in apocrine glands of normal and inflamed skin. Samples: Skin biopsies were taken from the axillary region of 15 volunteers (7 men and 8 women, aged 26-72 years) and from 10 patients with hidroadenitis suppurativa (5 men and 5 women, aged 25-55 years). No treatment had been received by the patients prior to biopsy. Results: The secretory portion of the apocrine glands showed cytoplasmic immunolabelling for all four cytokines. There was a variation in the staining intensity between resting glands and the active glands, which had cystically dilated, tall secretory cells with apical projections (Fig. 1, Table 1). The staining was more intense in the apical parts of the columnar cells and in the intact decapitated intraluminal material of the active glands than in the resting glands, but there was no immunolabelling of the diffuse homogeneous intraluminal material or the myoepithelial cells. No obvious differences were observed in the staining pattern between normal and inflamed skin with respect to the secretory parts of the apocrine glands. The occurrence of immunoreactive signals in the apocrine glands, especially in the columnar cells of the resting glands, may indicate that these cytokines are constitutive components. The intense immunolabelling of the columnar cells of the active glands, particularly the apical projections and the intact decapitated intraluminal material, suggests that the expression of these cytokines increases in the secretory phase. _______________________________________________________________________ Table 1 Cytokine staining intensity in the secretory part of apocrine glands of normal and inflamed skin (- negative, +/variable, + weak, ++ moderate, +++ intense) IL-1a IL1b IL6 TNFa Columnar cells Resting glands + + ++ ++ Active glands ++ ++ +++ +++ Apices of active cells ++ +++ +++ ++ Glandular lumen Intact decapitated apex +/++ +++ +++ Homogenous material __________________________________________________________________________ Conclusion: To our knowledge this is the first demonstration of these proinflammatory cytokines in apocrine glands. The apocrine glands may thus be regarded as an additional cytokine source in the skin. 1994 IL1 IL6 TNFa skin granuloma Ahmed.pdf Ahmed AA;Nordlind K;Schultzberg M;Lidén S interleukin-1 alpha- and beta-, interleukin-6- and tumour necrosis factor-alpha-like immunoreactivities in chronic granulomatous skin conditions. Acta Derm Venereol 1994 Nov;74(6):435-40 Aim:-The skin does not only constitute a simple mechanical barrier, but upon injury it may be activated to release cytokines from keratinocytes, actively participating in repair mechanisms and elimination of injurious agents. The 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 16 Annotations MSAB Database cellular distribution of IL-1 alpha, IL-1 beta, IL 6 and TNF-alpha in different chronic granulomatous skin conditions was investigated by immunohistochemistry in an attempt to understand their possible role in this reaction. Methods: Biopsy specimens were taken under local anaesthesia from lesional skin of untreated patients with cutaneous leishmaniasis (n=10), granuloma annulare (n=10), leprosy (n=6) and hidroadenitis (n=10). the duration of the clinical history was at least 6 months. Paraformaldehyde-fixed tissue of chronic granulomatous skin conditions, such as cutaneous leishmaniasis, granuloma annulare, leprosy and hidroadenitis, was investigated for the presence of interleukin-1 alpha-, interleukin-1 beta-, interleukin-6- and tumour necrosis factor-alpha-like immunoreactivities among the cellular infiltrates. Results: All specimens exhibited a heterogeneity in the morphology of the cells inflitrating the granulomas. Generally, a large number of infiltrating cells were encountered in the tissue surrounding the granulomatous mass, eg. close to sweat glands and blood vessels. There was a weak to strong cytoplasmic labelling of plasma cells for interleukin-6 and tumour necrosis factor-alpha at the periphery of the granulomatous mass and around the skin appendages. The interleukin-6-like immunoreactivity seemed to be correlated with the coarseness of the chromatin material of the cells, being more intense with coarse chromatin. The cytoplasmic labeling for interleukin-1 alpha and interleukin-1 beta in the plasma cells was less intense. Epitheloid, Langhans' giant cells and small round cells exhibited a weak to moderate cytoplasmic labelling for interleukin-1 alpha and interleukin-1 beta, whereas the staining intensity for interleukin-6 and tumour necrosis factor-alpha was weak to strong. In addition, there was staining of the stroma in the centre of granuloma with antisera against interleukin-1 alpha, interleukin-1 beta, interleukin-6 and tumour necrosis factor-alpha. This area contained few cells, suggesting that the granuloma was in a resolution process. Discussion/conclusion: A contribution of interleukin-6 and tumour necrosis factor-alpha to the granulomatous reaction, at least during the maintenance period, is suggested by the occurrence of these cytokines in the skin conditions studied. The findings are also consistent with a suggested role of B cells in the late stages of the granulomatous reaction. In addition, they are in line with the reported declining role of interleukin-1 in the maintenance of granuloma. In conclusion our findings suggest that IL-6, TNF alpha and plasma cells are active participants in the maintenance of granuloma. 1993 PDGF-receptor wound healing Acne Inversa Knaub.pdf Knaub S, Wlaschek M, Kirchberg K, et al. Abstract; differential regulation of the PDGF-recepto. Arch Dermatol Res. 1993 Nov; 285(1-2):64. Among other cytokines that have been implicated in wound healing, platelet-derived growth factor (PDGF) affects all phases of the healing process, including directed migration of wound edge associated fibroblasts into the wound bed with subsequent proliferation and enhanced matrix formation. Cellular responses to PDGF are dependent on the interaction of PDGF with its cell-surface receptors. However, even though two PDGF receptor subunits, termed alpha and beta, have previously been identified on a variety of cells in vitro, little is currently known regarding the regulated expression of the PDGF receptor subunits in vivo. Therefore we studied the expression of PDGF receptor subunits on human primary fibroblasts embedded into a three-dimensional collagen lattice which more closely represents the in vivo situation. In addition the appearence of PDGF receptor subunits was determined in human full thickness wounds at different time following radical surgical treatment of perigential (?perigenital) acne inversa. ..At day 6, 12 and 19 after surgical treatment induction of the alpha- as well as the beta-subunit was observed, whereas following reepithelialization of the restoration tissue at day 47, PDGF receptor expression was completely absent. ... The close interaction of fibroblasts with an increasingly organized extracellular matrix - as found during contraction of the collagen lattices and during cutaneous wound healing - appears to play an important role in the regulation of cellular responsiveness to growth factors by strictly controlling the synthesis and expression of the corresponding receptors. It remains to be elucidated whether downregulation of the PDGF receptor is altered in fibrotic and wound-healing disorders such as keloids and hypertrophic scar formation. 1990 HS a disease of follicular epithelium Yu.pdf Yu CC, Cook MG. Hidradenitis suppurativa: A disease of follicular epithelium, rather than apocrine glands. Br J Dermatol. 1990; 122(6):763-769. Examination of axillary skin specimens from 12 patients, compared to control specimens from cadavers with no previous history of HS Pathology of Hidradenitis Suppurativa Table 1 Histological features in HS (12 patients) FEATURE NO OF PATIENTS AFFECTED Cystic epithelium-lined structures (5/10 with disrupted epithelium) 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 17 Annotations MSAB Database (Half of these contained free hair shafts) Sinus tracts lined by squamous epithelium Chronic inflammation and scarring Acute inflammation No inflammation in apocrine glands Extensive inflammation (involving all dermal and appendageal elements) 10 4 12 8 7 4 1988 Glucose,clinical,microbiologic,immunologic features HS OLoughlin.pdf O'Loughlin S, Woods R, Kirke PN, Shanahan F, Byrne A, Drury MI. Hidradenitis suppurativa. glucose tolerance, clinical, microbiologic, and immunologic features and HLA frequencies in 27 patients. Arch Dermatol. 1988; 124(7):1043-1046. Study undertaken to evaluate 27 patients presenting to dermatology clinic with HS studied prospectively over a 3 year period-also glucose tolerance, HLA antigen frequency(1393 control group from a blood donor population), microbiologic studies and T and B lymphocyte peripheral populations assessed(control group healthy hospital staff). 14 male, 13 female subjects. 2/27 positive family history for HS Duration of disease ranged from 6 months to 19 years. 12/27 axilla/groin disease only. 10/27 had axilla, groin, perineum involvement. 3/27 had axilla/perneum disease only. 2/27 axilla disease only 9/27 had prior surgery for pilonidal sinus. 8/27 obese. Additional medical disorders: 1/27, diabetes mellitus, celiac disease and ulcerative colitis 1/27 recurring cutaneous candidiasis and alopecia areata 1/27 reactive anxiety, depression and hayfever 2/27 positive family history for Diabetes Mellitus (including one with family history of HS) Hs severity graded 0,1,2,3, representing absent on examination or mild, moderate, severe. Acne graded(Cunliffe)_______________________________________ SEVERITY No. of patients(n=27) Absent 1 Mild 12 Moderate 10 Severe 4 __________________________________________ ACNE SCORE (Cunliffe scale 1981) No OF PATIENTS(n=27) 0 18 1 4 2 4 3 1 ________________________________________________ GLUCOSE TOLERANCE ABNORMAL No of patients(N=27) Diabetes Mellitus 2 (Absent or grade 1 HS, both absent acne) Impaired GT 4 (1/4 grade 1 HS, 2/4 Grade 2 HS,1/4 grade3 HS,1/4 Grade 2 Acne) Flat GT curve 1 (Grade 3 HS, Grade 3 Acne) NOTES: 4/27 or 16% incidence of impaired Glucose tolerance compared to expected incidence in general population-1-2% (unpublished data, 1985). Two DM patients had the HLA-B8 antigen, which is more common diabetics. Two impaired glucose tolerance case also had the HLA B8 antigen Flat glucose tolernace curve is thought to be common (unpublished data, 1985) ________________________________________________________________________________ Samples from following sites taken: Axilla n=20, Groin n=7,perneum n=5) MICROBIOLOGIC FINDINGS Isolates found(n=32) 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 18 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database Staphylococcus Epidermis 15 Staphylococcus pyogenes 5 Anaerobic Streptococci 5 Proteus Mirabilis 3 Diptheroid 2 A-Hemolytic Streptococci 1 Bacteroides Fragilis 1 TOTAL SAMPLES YIELDING CULTURE 100% Note: Strep Milleri sought but not isolated. __________________________________________________________________________________ HLA TYPING AND LYMPHOCYTE MARKERS 25 HLA and B frequencies were similar to that found in the blood donor population HLA-A19 however was significantly lower in HS patients (P<.01) Median value of percentage of Tcells was the same (65%) as control subjects(10 men and 10 women) Median value for percentage of Bcells was significantly lower in HS patients (10%) compared to controls (12.5%), however each patients Bcell numbers were within normal ranges. Of 14/27 patients who had moderate to severe HS seven had markedly reduced Tcells, and these had a higher frequency of HLA A1 (71%) and HLA B8 (57%) and the A1/B8 combination (57%) compared with the other HS patients and control group. Only the A1/B8 linkage comparison between the seven patients (57%) with low Tcells and the other 20 patients (10%) was statistically significant (Fishers exact test; P=.05). For the 27 patients with HS we examined the association between HLA antigens and the distribution of T-cells. Of 102 comparisons made, the only significant association with a low percentage of T cells(,50%) was the presence of the A1/B8 combination. This could be a chance association. CONCLUSION: from our preliminary findings we suggest a comprehensive study is warranted to evaluate helper, cytotoxic, inducer and suppressor T-lymphocyte populations, acute phase proteins, and humoral aspects of host defense mechanisms in patients with HS before and after treatment. 1988 Bacteriology Perineal HS Highet.pdf Highet AS, Warren RE, Weekes AJ. Bacteriology and antibiotic treatment of perineal suppurative hidradenitis. Arch Dermatol. 1988 Jul;124(7):1047-51. AIm: In three patients with perineal suppurativa hidradenitis, we found strong circumstantial evidence for a significant pathogenic role for Streptococcus Milleri. We describe our clinical and bacteriologic findings in 32 patients with perineal disease with particular attention to 1. Correlation between the prescence of individual organisms and the clinical severity of the disease and 2. Clinical and bacteriologic responses to antibiotic therapy. Patients/methods: Lesional discharge obtained for bacteriologic exam from 32 patients with a clinical diagnosis of chronic recurrent suppurative hidradenitis of the perineal region. We use perineal to include genitalia, groin, pubic area, upper thighs, buttocks and perineal skin. There were 13 men (mean age 39 years, range 23 to 64 years) and 19 women (mean age 34 years, range 21 to 67 years). Data from three previously described patients were included. At each visit a clinical assessment was made of overall disease severity, taking account of pain, tenderness, and discharge, and recorded on a six-point scale (0-5). Patients with disease activity of grade 2 or higher were usually given antibiotic therapy after the specimen was obtained for culture, but before the results were available and reviewed after one to four weeks. All patients were encouraged to attend immediately if the disease became more active. The number of examinations per patient varied between one and 22, the total number of examinations was 181. _____________________________________________________________ Table 3 Classification of Organisms isolated ORGANISM # Patients Total # Isolates Streptococcus Milleri 21 87 Staphylococcus Aureus 14 25 Aneorobic Streptococci 12 32 Bacteroides Species 10 26 Gorp B streptococci 5 7 Group D streptococci 8 9 Proteus species 9 42 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 19 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database Coliform bacteria 10 19 Pseudomonas species 1 1 _____________________________________________________________ Streptococcus milleri was isolated more frequently from those patients who were examined most often: these pateints tended to be more severely affected. S milleri, whose presence was significantly associated with disease activity and whose disappearance significantly correlated with clinical improvement. Unexpected Treatment Failures: Lack of clinical improvement when the patient was given an antibiotic to which the organism was reported to be sensitive occurred 32 times including 15 cases where none of the other three main bacterial groups was isolated. Of these 15, six failures were of penicillins against S milleri and five were of tetracyclines (doxycycline) against S milleri. Most frequently examined patients: Of the six most frequently examined (more severely and persistently affected by HS) there were five with persistent S milleri infection. Resistance to tetracyclines developed in four cases, and resistance to erythomycin in four, including three cases with resistance to both drugs. Bacterially negative patients: 6 patients yielded none of the bacteria listed. One was examined five times, on twice, and four only once. In 6 of these 11 cases, antibiotic treatment which was commenced after specimen collection wa followed by clinical improvement. One patient deteriorated on clindamycin. For one of these patients on three occasions no treatment was given: on the first examination she improved, second examination she deteriorated, third she was unavailable for follow-up. Comment: Our understanding of the pathogenesis of HS is incomplete. Streptococcus milleri may be missed in routine laboratory tests unless specifically sought. The value of erythomycin and tetracyclines against S milleri is clouded in this chronic relapsing condition by the frequent appearance of resistance to both drugs when repeated courses of treatment are given. This is an unusual double resistance and may suggest either a mixed population or plasmid transfer from other members of the flora. resistance was a major problem for the more severely affected cases. In our first article we described the possible relevance of S Milleri in normal carriage sites (mouth, throat, vagina and bowel) in the management of patients with HS ifected with this organism. We have, however, found no correlation between its presence in carriage sites and its presence in lesions (data not presented). CONCLUSION: About a quarter of our cases achieved convincing clinical relief without immediate relapse. Many patients, however do not improve significantly on treatment that appears to be bacteriologically appropriate and that in a few patients no presumptive pathognes can be found on repeated examination. We emphasize that we do not consider bacterial infection to be the sole or primary abnormality in suppurative hidradenitis, but regard it as a common secondary complicating factor which is sometimes amenable to simple oral therapy. 1986 (Abstract) cellular and humoral immunity acne Schmidt Schmidt JB, Pinzker H, Eibl M. Abstract of Parameters of cellular and humoral immunity in patients with severe acne. [Article in German] Z Hautkr. 1986 Oct 1;61(19):1397-404. Severe forms of acne, characterized by predominance of inflammatory reactions and persistence of the disease for years, still present therapeutic problems. The purpose of our study was to investigate the role of the immune system in severe persisting forms of acne by means of determination of cellular and humoral immunobiological parameters. The study was performed on 52 patients (47 males and 5 females) having suffered from severe acne for six years on an average, including papulopustular acne grade IV resistant to therapy,nodulocystic acne, conglobate acne, and acne tetrade. The results were compared with those of 52 healthy controls of the same age showing no inflammatory diseases, who were tested on the same day. In 56% of the acne patients, one or more parameters showed pathological values, while in nodulocystic and conglobate acne there were similar results with regard to cellular defects and acute phase reactants. The lymphocytic proliferation induced by mitogens was significantly decreased in 35% of the acne patients. We assume that the immunodeficiency observed in these patients may be mainly secondary; however, it may contribute to the perpetuance of the disease and its resistance to therapy. 1982 Evidence for a bactericidal defect Ginder.pdf Ginder PA, Ousley M, Hinthorn D, Liu C, Abdou NI. Hidradenitis suppurativa: evidence for a bactericidal defect correctable by cholinergic agonist in vitro and in vivo. J Clin Immunol. 1982 Jul;2(3):237-41. Case report- 70 y.o. male 10 year history HS of axilla and perineum. Extensive leukocyte function studies were undertaken, disclosing normal phagocytosis and chemotaxis, but defective bactericidal function. This defect was correctable in vitro by the addition of cholinergic agonists. After one month on bethanechol chloride, 10mg tds, 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 20 Annotations MSAB Database changed to 50 mg tds in second week, there was striking improvement. Pain and swelling decreased, purulent drainage stopped and sinus tracts began to heal. The patient was continued on bethanechol chloride and was followed for approximately 14 months without evidence of recurrences of the abscesses. Bactericidal Activity: Leukocytes from the HS patient had decreased bactericidal activity. The number of E. coli colonies present in the disrupted HS cells was over 20 times greater than that from normal controls (Table I). This defect could be corrected in vitro by incubation with 10 -5 M carbachol, which decreased the number of colonies relative to that of controls, Leukocytes from the patient tested 2, 4, 6, and 9 months after the institution of in vivo treatment with 150 mg bethanechol daily were as effective as normal cells in killing E. coli (Table I). When the patient discontinued therapy for several months on his own initiative, suppurating lesions recurred. There was healing upon the reinstitution of bethane-chol. Unfortunately, we did not have the opportunity to do in vitro studies during the period off therapy. Cyclic Nucleotide Levels: The patient's leukocyte cyclic AMP levels before or after in vitro treatment with carbachol were not significantly different from those of leukocytes of three normal controls (Table II). In contrast, cyclic GMP was very low in the cells from the HS patient, when compared to levels of normal cells. Cyclic GMP increased to almost-normal levels after in vitro treatment of the HS cells with carbachol or in vivo treatment of the patient with bethanechol chloride Discussion: The observed neutrophil abnormalities in our patient were probably not secondary to HS per se, since we cannot demonstrate similar defects in three other patients with HS (unpublished observations). We cannot exclude the possibility that these cellular functional defects could be an epiphenomenon of chronic inflammation and not a pathologic entity. There are unanswered questions about host-defense abnormalities such as those seen in this patient with HS. Our patient was anergic and had a markedly depressed in vitro blastogenic response to phytohemagglutinin despite normal numbers of T cells. The mechanisms responsible for the low levels of cyclic GMP and its relationship to anergy, poor mitogen responses, and a phagocytic cell abnormality suggest a broad immune defect. Chronic indolent infection may cause blunting of immune function as described in leprosy (16). The imbalance of intracellular levels of cyclic nucleotides could result in enhanced prostaglandin E2 release. The latter could then suppress T-cell proliferationfunction and the bactericidal function of phagocytic cells (17). Although these features require further work, it is clear that cholinergic agonists were helpful in the therapy of the patient reported in this paper and merit further evaluation in the treatment of HS or in other cases of neutrophil killing defects. 1979 Anaerobic Axillary Abcess Leach.pdf Leach RD, Eykyn SJ, Phillips I, Corrin B, Taylor EA. Anaerobic axillary abscess. Br Med J. 1979 Jul 7;2(6181):5-7. The isolation of anaerobes in our laboratory from a patient with HS led us to review all cases of axillary abscess. Pus from each axillary abscess was submitted in a sterile container or on a swab. Patients: Fifty-two patients with axillary abscesses were seen during two years. 15 men, 37 women, mean age at presentation 29 years. Staphylococcus aureus was isolated from 34, anaerobic bacteria from 12, and skin flora from five; in one case the pus was sterile. Patients with HS: Seven patients with hidradenitis suppurativa had recurrent infection with abscess formation, which was bilateral in three. Anaerobes were isolated in five of these cases and skin flora alone in two. Anaerobes are secondary invaders in this condition, Histology: Three HS samples showed keratin plugging of the pilosebaceous follicles Discussion: (In comparison to axillary abscesses), HS presents a quite different clinical picture of multiple small abscesses with undermining and induration of axillary skin. 1977 Host Defence Mechanisms Dvorak.pdf Dvorak VC, Root RK, MacGregor RR. Host-defense mechanisms in hidradenitis suppurativa. Arch Dermatol. 1977; 113(4):450-453. Host-defense mechanisms were studied in seven patients with active hidradenitis suppurativa (HS). Interesting note from introduction citing Steiner,K;Grayson LD. HS of the adult and its management. Arch Dermatol 71:205-211,1955. "According to the pathologic findings, HS has been divided into acute (AHS) and chronic (CHS) forms; AHS lacks sinus tracts and hypertrophic scarring. Staphylococcus Aureus is the most frequent organism found in AHS, while CHS has infection secondary to Gram-negative organisms as frequently as it has with S aureus. Infection is probably a secondary event of HS and not its cause" Defects in host mechanisms have been suspected but not systematically investigated in patients with HS. To resolve this question we studied cellular, humoral, and granulocyte defenses in seven patients with Chronic HS. At the time of the study all patients had suppurating lesions. Controls for the investigation consisted of normal laboratory personnel. TABLE 1 PATIENTS CHARACTERISTICS PATIENT AGE YR SEX HS DISTRIBUTION DURATION UNDERLYING ILLNESS 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 21 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database 1 26 M Generalized 7 2 39 F Generalized 15 3 4 30 32 M F Generalized Axilla 10 17 5 6 7 48 27 33 F M F Axilla Generalized Generalized 8 10 5 Pilonidal cyst 2yr prior to onset; Psoriasis 3yr prior to onset Mild obesity, recurrent thrombophlebitis; Positive family history for HS Four pilonidal cysts prior to onset; acne Obesity;DM;hypercholestorlemia; Pilonidal Cyst 2 yr after onset Obesity Down s syndrome Obesity; alcoholism; abnormal GTT; Sister of patient 2(family history) Chemotaxis,Phagocytosis-n, Intracellular killing of ingested bacteria-no substantial difference to controls. Granuloycte adherence increased in two patients (1 and 6). Complement raised in all patients. IgG and IgA elevated in patient 6 only (Downs Syndrome) 1958 Clinical and Pathologic Study Perianal HS Anderson.pdf Anderson M,Jr, Dockerty M. Perianal hidradenitis suppurativa; a clinical and pathologic study. Dis Colon Rectum. 1958; 1(1):23-31. "HS is a chronic inflammatory condition affecting the skin and subcutaneous tissues in those regions in which apocrine sweat glands are said to be found, namely the axilla, about the areola of the breast, in the inguinal regions, about the external genitalia and in the perianl region. Late in its course, the disease is characterized by the formation of subcutaneous abscesses, fistulas and sinuses, and by cutaneous scarring....this disease has not attracted widespread attention among physicians. Most of the reports concerning hidradenitis represent impressions based on a relatively narrow clinical experience. AIM: Present the results of a clinical and pathologic study of a sizeable group of cases of hidradenitis, comparing our findings with comparable data gleaned from the literature. ETIOLOGY: Closure of the pores has been suggested as a contributing factor, but Hurley and Shelley demonstrated experimentally that such closure does not produce disease of the apocrine glands. Most investigators believe that specific organisms have no significance in the production of hidradenitis.(Cites:Hurley,HJ Jr.;Shelley,WB. Apocrine sweat retention in man. I. Experimental production of asymptomatic form. J Invest Dermatol. 1954 May;22(5):397404. does not cite Shelley and Cahn 1955). PATHOGENESIS: Nearly all writers agree that the infection is exogenous, but some disagreement exists whether the apocrine glands are involved primarily or secondarily. Some investigators consider that the causative agent enters through the hair follicle and the excretory duct of the apocrine gland, whereas others contend that the apocrine glands are involved only by contiguity. OTHER-has interesting disease description, differential diagnosis of early and late stage lesions and summarizes historical treatments used..antibiotics: little evidence exists to attest to their efficacy -use of roentgen therapy for hidradenitis was widespread in the 1920's to 1930's. It is still employed but its use ordinarily is restricted to cases of early and mild disease -it is said one should not delay employment of radical surgical treatment, since a sympathetic relationship appears to exist among the various sites involved by this disease. One might prevent the occurrence of infection in one site by surgical removal of seriously involved tissue in another location. MATERIAL AND METHODS-Study of records of 117 patients seen at the Mayo Clinic during the decade beginning Jan 1 1940, who were considered to have HS of the perianal region. 64/117 were treated surgically at the clinic. This group of 64 patients provided material for pathologic study. From the preserved gross specimens available from these 64 patients, a total of 261 sections were prepared and stained with hematoxylin and eosin. CLINICAL FINDINGS:65% of patients were male, 35% female. Age at onset- 26.5% in second decade, almost as great in the third and fourth decade, 4.5% onset during first decade, 17% onset after the fourth decade. Oldest patient 68 at onset. SITES OF INVOLVEMENT: Perianal region involved in 1 out of 6 patients who had HS, perianal region involved alone in one of every 13 patients. In this series it was not unusual for hidradenitis to be found in regions other than those which for so long a time have been called typical. Cases were found in this study in which the posterior part of 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 22 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database the neck, the scalp, shoulders, extremities, abdomen or face was involved synchronously or metacronously by the disease. CO-EXISTING DISEASE: -only 1 case of unconfirmed anal fistula (patient refused surgery) authors dispute that anal fistula due to HS is common -Pilonidal disease in 16/117 patients -Acne present in at least 30%-some common etiologic factor might be present in acne and hidradenitis -endocrine effect suggested by only 10 cases, 5 cases of pre-menstrual exacerbation, 2 cases onset of disease coincided with menarche, effect of pregnancy on HS variable PATHOLOGIC FINDINGS: Most frequently noted gross finding was that of scarring and roughening of the epidermis. Dermal and subcutaneuos fibrosis was characteristic. Microscopic:261 sections from 64 patients revealed eccrine glands present in 59, wheras typical apocrine glands were identifiable in only seven. Since some sections were taken from the least involved zones in each case, complete destruction of the apocrine glands by the inflammatory process would not appear to explain the infrequency with which apocrine glands were found. Inflammatory cellular exudate conisted primarily of plasma cells, with some lymphocytes and fixed connective-tissue cells in nearly all cases studied-which was considered to be consistent with the chronicity of the clinical symptoms and signs. The distribution of the cellular exudate also similar in most cases. Perivascular infiltration was noted in the tissues of 42 of the 64 surgical patients, and giant cells were noted in these zones of diffuse inflammation in 12 instances. Cellular exudate also was noted rather frequently just beneath the epidermis, as well as in the tissue spaces or small lymphatic spaces. 2 cases malignant change in the form of squamous cell epithelioma,-eccrine sweat glands and inflammatory changes typical to HS noted in addition to malignant tumour-1 case HS present for 25 years, 35 years in the other PATHOGENESIS OF HS -findings in our study indicate that the apocrine sweat glands are infected secondarily rather than primarily. TREATMENTFollow up sought on 64 patients, information obtained from 38 patients, 26 lost to followup Surgical treatment in most cases consisted of excision of the sited involved by hidradenitis. This was usually accompanied in one stage, but two stage procedures were used in 15 cases and three stage operations were necessary in six instances. 8/38 (21% of those followed up) had no subsequent symptoms. Follow up time 8 years or more. 12/38 (32%) had mild symptoms after operation but required no further treatment. Follow up time 30 for more than 9 years, 1 patient two months, 1 patient 3years 17/38 (45%) required further treatment after surgical intevention at the clinic-consisting of one or more further surgical procedures in almost all cases 1/38 cases died two years after excision of a grade 2 squamous cell epithioloma of the buttock-this patient had a 32 year hx of HS SUMMARY AND CONCLUSIONS: Perianal HS occurs in males nearly twice as often as it does in females. Anal fistula secondary to hidradenitis is rare. Malignant degeneration occasionally occurs as a complication of longstanding hidradenitis. Apocrine sweat gland.involvement appears to be coincidental. The cause and pathogenesis of HS are not well understood. The disease apparently has some relationship to endocrine activity, and it appears likely that common etiologic factors are present in hidradenitis and acne. Other than the presence of apocrine glands, local conditions exist that might help to explain the typical distribution of HS. Among these are the frequent apposition of cutaneous surfaces, escessive local moisture, heat and friction, and the difficulty of maintaining cleanliness in the regions involved. The treatment of hidrdenitis of the perianal region is somewhat discouraging because many patients continue to experience recurrent activity of the disease even after careful excision of all active sites. To attempt to excise all of the skin in which recurrent activity might appear is out of the question, so one must be content to explain that future periodic surgical care is a possibility. Until the cause and pathogenesis of the disease are better understood, it is doubtful that the progmosis will improve. GENETICS 2006 HS case report chromosomal localization GAO.pdf 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 23 Annotations MSAB Database Gao M, Wang P, Cui Y, et al. Inversa acne (hidradenitis suppurativa): A case report and identification of the locus at chromosome 1p21.1-1q25.3. J Invest Dermatol. 2006 Mar; Advance Online Publication(Mar 16) We performed a genome-wide scan in a four-generation Chinese family to map the chromosome location of the responsible gene. Study participants: A four-generation family (Figure 1) from Anhui province of China with the inversa acne features was recruited for this study. It showed an autosomal dominant inheritance pattern. The proband, individual III:8, is a 34-year-old male, and at the age of 12 years, he presented the red papules on his face and neck. In the recent 2 years, the disease had gradually become prominent on his face, neck, limbs, truck, groin, especially on the scalp, axillae, and buttocks, and the lesions developed inflammatory papules, painful nodules, pustules, sinuses, and abscesses (Figure 2a–d). The eruptions were remarkable after alcohol intake. The ages of onset in this family are between 10 and 20 years. After obtaining informed consent from all the participants, blood samples were collected from available family members and genomic DNAs were extracted from peripheral blood by use of a blood kit (Qiagen Inc., Hilden, Germany). This study was approved by Anhui Medical Institutional Review Board and conducted according to the Declaration of Helsinki Principles. In this study, we collected a large inversa acne pedigree and performed a genome-wide scan. The proband and his brother (III:10) were affected by acne conglobata, inversa acne and dissecting cellulitis of the scalp, but other affected members mainly have mild acne inversa. The loci of the diseases have not been genetically mapped. We performed a genome-wide scan in this family and found that the twopoint maximum LOD score obtained was 3.26 with markers D1S2624. We positioned the locus upper to D1S248 and lower boundaries to D1S2711. This 73Mb critical region contained about 886 genes, including about 395 known genes, a large number of predicted genes, and numerous expressed sequence tags. There are many possible candidates in this region. This study firstly identified a novel locus for inversa acne on chromosome 1p21.1–1q25.3 in a large Chinese family. Because this region is still too wide to find the disease gene, the further studies are continuing to collect new families to confirm and fine mapping this locus. It will be a starting point towards understanding the molecular mechanisms of this disease. ACKNOWLEDGMENTS This work was funded by the grants from the Chinese High Tech Program (2003AA227030), the Shanghai Science and Technology Committee (03DJ14008), and the Chinese Ministry of Education (2003). We are most grateful to the family members who participated in this study. 2006 ABSTRACT Clinical and cytogenetic analysis of 46 HS cases Mehta Mehta T, Shah N, Sheth J, Nath S, Radhakrishna U. Abstract: Clinical and cytogenetic analysis of forty six hidradenitis suppurativa cases from gujarat india. Unpublished abstract presented to the HSF for research purposes. 2006 Mar. 2005 Lecture abstract-comparing Interleukin polymorphisms Acne HS Koreck.pdf Koreck A, Szell M, Kis K, et al. William J Cunliffe Scientific Awards S46.5 Polymorphisms of Interleukin-1 Receptor Antagonist, Toll-Like Receptor 2 and 4 Genes in Patients with Acne 12-16 October, 2005 EADV 14th Congress London <http://www.eadv2005.com>. EADV, 2005. We investigated the allelic distribution of the variable number of tandem repeat (VNTR) polymorphism of interleukin1 receptor antagonist (IL-1RN), and single nucleotide polymorphisms (SNP) of TLR2 gene resulting in the amino acid changes Arg677Trp, and Arg753Gln as well as SNP’s of TLR4 gene resulting in the amino acid changes Asp299Gly, and Thr399Ile in patients with acne (n=47), acne inversa (n=18) and in a control population (n=16). None of the studied TLR2 polymorphisms were detected either in patients or healthy individuals and no difference was found in the prevalence of TLR4 alleles among the three groups. The frequency of the two repeat allele of IL-1RN (allele 2) was slightly increased among acne patients compared both to controls and to acne inversa patients, moreover, among the studied groups, only acne patients were homozygotes for allele 2, and 4 of these 6 homozygotes suffered from acne conglobata. 2005 KID syndrome HS Maintz.pdf Maintz L, Betz R, Allam J, et al. Keratitis-ichthyosis-deafness syndrome in association with follicular occlusion triad. Eur J Dermatol. 2005 Sep-Oct; 15(5):347-52. case study 2005 Abstract Follicular Occlusion Triad (English transl. and Chinese) Xu.pdf Xu W., Zhao J, Zhao H, Gu F, Huang X.. Follicular Occlusion Triad: Abstract of A Case Report and Pedigree Analysis Chinese Journal Of Dermatology 2005 Vol.38 No.3 P.157-159 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 24 Annotations MSAB Database http://www.wanfangdata.com.cn/qikan/periodical.Articles/zhpf/zhpf2005/0503/050308.htm (In Chinese and English transl. by Dr. Jihai Shi). A family of 13 affected individuals is reported. 2004 KID syndrome connexin 26 defect HS Montgomery.pdf Montgomery JR, White TW, Martin BL, Turner ML, Holland SM. A novel connexin 26 gene mutation associated with features of the keratitis-ichthyosis-deafness syndrome and the follicular occlusion triad. J Am Acad Dermatol. 2004; 51(3):377-382. case study 2004 HS DDD epidermal cysts Loo.pdf Loo WJ, Rytina E, Todd PM. Hidradenitis suppurativa, dowling-degos and multiple epidermal cysts: A new follicular occlusion triad. Clin Exp Dermatol. 2004; 29(6):622-624. case study 49 year old female multiple recurring nodules and abcesses for 11 years. 8 relatives from her fathers family have similar although milder skin lesions. Also noted macular pigmentation in a flexural distribution and around her waistline. Histopathologic diagnosis of HS and Dowling Degos Disease. Patient and family referred for investigation with regards to any underlying genetic defect for their condition. 2004 HS and the Van der Woude Syndrome Dissemond.pdf Dissemond J, Haberer D, Franckson T, Hillen U. The van der woude syndrome: A case report and review of the literature. J Eur Acad Dermatol Venereol. 2004; 18(5):611-613. case study 2003 PRESENTATION ABSTRACT Autosomal Dominant HS large Indian family Radhakrishna.pdf Radhakrishna U, Mehta TY, Solanki JV, Rao UC. An autosomal dominant hidradenitis suppurativa in a large Indian family.53rd Annual Meeting of The American Society of Human Genetics Los Angeles, California.<http://genetics.faseb.org>. Accessed 4/24/2005. FASEB, USA, 2003. genetic study, 20 affected with HS in one large family 2002 Cytokeratin expression Pilonidal Sinus Kurokawa.pdf Kurokawa I, Nishijima S, Suzuki K, et al. Cytokeratin expression in pilonidal sinus. Br J Dermatol. 2002; 146(3):409413. The aim of our study was to clarify immunohistochemically CK expression in PS, and to compare that with the previous study in HS. Samples of PS were surgically excised from nine male patients. The age of the patients ranged from 22 to 46 years. All specimens were obtained from the buttock. As a whole, CK expression in PS was comparable with that in HS as previously reported.7 Type A epithelium resembles infundibular epithelium because of the presence of CK1 and 10 in the suprabasal layers and CK14 in the basal layer, and the absence of CK16 and 19. However, CK17 was not found in type A epithelium. In the infundibulum, CK17 was found in the suprabasal layers of normal skin. CK17, spatial keratin, has a function related to the three-dimensional cytoskeleton structure.12The absence of CK17 in type A epithelium may reflect a fragile follicular structure, resulting in the rupture of the follicle, which subsequently forms a subcutaneous abscess. From the perspective of CK expression, CK expression in PS was comparable with that in HS, which supports the concept of Plewig et al.2,3 who have advocated that PS belongs in the follicular occlusion tetrad (acne tetrad) category. 2002 Cytokeratin expression HS Kurokawa.pdf Kurokawa I, Nishijima S, Kusumoto K, Senzaki H, Shikata N, Tsubura A. Immunohistochemical study of cytokeratins in hidradenitis suppurativa (acne inversa). J Int Med Res. 2002; 30(2):131-136. Hidradenitis suppurativa (HS), also called acne inversa,1 is characterized by the formation of undermined draining sinus tracts, resulting in dissecting cellulitis. More common in men, HS occurs in adults and lesions can be found in the axillae, buttocks, perianal region or thighs.1, Our findings relating to CK expression in normal skin were consistent with previous studies.12 – 17 Kurzen et al.20 reported a detailed study in the draining sinus in HS. These types are not comparable to our epithelial classification, although type A in our study was similar to their type I. The overlying epidermis in HS lesions therefore has a more undifferentiated hyperproliferative state than that found in the normal epidermis. In some cases, CK19 was found in the 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 25 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database basal layer of the epidermis, facing the dermal papilla, and CK19 expression is associated with pre-malignant change.10 Further studies are necessary to evaluate cytokeratin expression by accumulating additional cases of squamous cell carcinoma in HS. 2001 Swedish HLA B DR Frequency HS Lapins.pdf Lapins J, Olerup O, Emtestam L. No human leukocyte antigen-A, -B or -DR association in swedish patients with hidradenitis suppurativa. Acta Dermatovenereologica. 2001; 81(1):28-30. In a previous study using serological tissue-typing techniques, no significant increases in the HLA-A and -B specificities were found in 27 Irish patients with HS (10). The aim of this study was to determine the frequencies of HLA-A and -B alleles using genomic tissue-typing methods in Swedish patients with HS. DR typing was also included, for the first time to our knowledge, in order to assess HLA class II locus association in HS. Study subjects: Forty-two unrelated Swedish patients (mean age 44 years; range 26 ± 62 years; 40 women; 2 men), with a history of HS were included in this study. All patients were stage II cases, according to clinical staging adapted from Hurley, i.e. recurrent abscesses with tract formation and cicatrization and single or multiple widely separated lesions. We included no cases of concurrent diseases with fistulizing tendencies, such as regional enterocolitis, ulcerous colitis or rheumatoid arthritis. The patients were otherwise healthy. A group of 250 healthy, randomly selected Swedish people was used for comparison. All subjects gave their informed consent and the study was approved by the local Ethics Committee at Karolinska Institutet. RESULTS Patients and controls were studied for the frequencies of HLA-A, -B and -DR specificities using PCR amplification with SSP. The distributions of all presently known alleles for HLA-A, -B and DR were similar in patients with HS and controls (Table I). Moreover, normal frequencies of the HLA-A1 and -B8 types were found in patients with HS. In conclusion, no HLA association was observed in Swedish patients with HS. Thus the notion that specific HLA antigens might confer genetic susceptibility to HS was not supported and remains questionable. 2000 Clinical Genetics of HS revisited Von Der Werth.pdf von der Werth JM, Williams HC, Raeburn JA. The clinical genetics of hidradenitis suppurativa revisited. Br J Dermatol. 2000; 142(5):947-953. Von der Werth and Williams 2000 Clinical genetics 28 HS patients, 18 possibly affected with HS but not clinically proven, both Peter Mortimer and Gregor Jemec helped provide a diagnostic method for the study. This study on the pathogenesis of HS validates and reproduces prior study results which demonstrate that a form of HS occurs in familes. Samples from patients within these families were assessed in a laboratory, in order to search for a molecular genetic abnormality. 1999 Epithelial differentiation sinus tracts HS Kurzen.pdf Kurzen H, Jung EG, Hartschuh W, Moll I, Franke WW, Moll R. Forms of epithelial differentiation of draining sinus in acne inversa (hidradenitis suppurativa). Br J Dermatol. 1999; 141(2):231-239. We examined the epithelial differentiation of draining sinus in acne inversa using monospecific CK protein antibodies and desmosomal protein antibodies in order to study the influence of chronic inflammation and hyperproliferation on cytoskeletal and desmosomal proteins. Materials and methods: We examined skin samples from 15 patients obtained during surgical removal of chronically inflamed lesions from the axilla and the groin. The interesting point is that all three types of sinus epithelium identifed by our marker studies apparently lack a normal counterpart and thus can be regarded as pathological phenotypes. In conclusion, the epithelial lining of the draining sinus in acne inversa is heterogeneous and comprises three different phenotypes of pathologically altered stratifed squamous epithelia de®ned by the expression of special combinations of CKs and desmosomal proteins. 1985 Family study HS Fitzsimmons.pdf Fitzsimmons JS, Guilbert PR. A family study of hidradenitis suppurativa. J Med Genet. 1985; 22(5):367-373. (Refer to next study, same study, reworded, different publications) 1985 Evidence of Genetic Factors HS Fitzsimmons.pdf 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 26 Annotations MSAB Database Fitzsimmons JS, Guilbert PR, Fitzsimmons EM. Evidence of genetic factors in hidradenitis suppurativa. Br J Dermatol. 1985; 113(1):1-8. Summary: The possibility of genetic factors in this disorder were previously noted. There is a lack of detailed family studies and in view of the ease with which the first three families were identified, further family studies were undertaken. Methods: Twenty-six probands suffering from hidradenitis suppurativa were identified from a busy general hospital providing specialist plastic surgical services for the East Midlands, City Hospital, Nottingham Hospital Activity Analysis (H.A.A.) records for the period 1980-83 and by direct referral from hospital specialists over a 6-month period in 1983-84. Affected individuals were also referred from hospital specialties including dermatology and general surgery in the South Trent region. Patients attending GP.s and other outpatient specialties were not included. HS was diagnosed on the basis of recurrent suppurativa cicatrizing lesions of apocrine gland bearing areas of the skin, primarily affecting the axillae and anogenital area. Acne Conglobata was diagnosed on the basis of abscesses and scarring affecting predominantly the back, chest and buttocks. Family pedigree information was collected by domiciliary visits or hospital interviews. In 31 of the 62 affected the diagnosis was made by a consultant dermatologist; 17 were examined by the authors; 14 declined to be visted or examined and the diagnosis was presumed on patient and family doctor information. Results From the 26 probands investigation of their families eventually confirmed a total of 62 affected individuals from 23 families. Problems of identification including variable age of onset and psycho-social factors were found and may be responsible for false-negative family histories. _____________________________________ TABLE 1 In eleven familes A-K 48 (35 females, 13 males) In three families a-c 3 In nine families 1-9 9 In two families (uncooperative) 2 Total (22 males 40 females) 62 ______________________________________ Families A-K In II families there was evidence in favour of single gene or Mendelian inheritance. The disease is transmitted vertically through three generations in five families and through two in six families. In family D there is male to male transmission, and in family B identical twins were both affected. Families a-c There was a history of the disease in a parent of the proband in two families and in a cousin of the proband in the third. One of these families appears to demonstrate a further instance of male to male transmission. Families 1-9 In nine families there was no confirmatory evidence of the disease in any other individuals at the time of the investigation. (However, this group initially included a further family where it was presumed there was only one affected individual. Subsequent contact with a knowledgeable and cooperative key person confirmed that the probands mother and sister both suffered from the disease as did their mother. This is family K in the first group). Discussion Familial clustering of disease is well recognized and does not necessarily imply a genetic origin. However the number of affected individuals in the first eleven families and the mode of transmission through several generations is consistent with single-gene dominant inheritance. The male to male transmission demonstrated in family D and possibly also in family b would appear to confirm autosomal and not X-linked dominant inheritance. In families A-K the number of affected is 34%, less than the expected 50% to demonstrate dominant inheritance. 46/62 affected probands developed the disease in their late teens or early adult life and 16 between the ages of 30 and 40 years. There were at least 40 individuals who were first degree relatives of the probands in the groups A-K and were under 20 years. This fact coupled with incomplete ascertainment and variable penetrance may distort the expected proportion of affected individuals. Psychosocial factors were noted to be important and a number of females were reluctant to admit to the disease, particularly vulval disease. In some cases, the presence of the condition was concealed from parents and close relatives. These families (1-9) will require periodic examination. It is possible that HS may have several causes, with genetic factors being important in some families but not in others. On occasions, multifactorial inheritance may 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 27 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database mimic mendelism but the number of affected individuals in families A-K considerably exceeds that expected on a multifactorial model. Acne: Only 8 of the affected individuals in this study had acne conglobata, five from family A. This was an unusual pedigree. There was no historical or clinical evidence of an increased incidence of severe acne vulgaris in the remaining patients. ____________________________________________ TABLE 2 HS FREQUENCY in first degree relatives of probands RELATIONSHIP Siblings Parents Children TOTAL No. Affected % Affected EXPECTED 29 10 35% 22 8 36% 50% 19 6 31% --------70 24 34.3% _______________________________________________ Conclusion: Most individuals with HS consider this condition to be an extremely burdensome one, although there is an obvious variation in clinical severity. This coupled with the apparent familial implications make it important to enquire and examine relatives of individuals found to be suffering from the disease. 1984 Familial HS Fitzsimmons.pdf Fitzsimmons JS, Fitzsimmons EM, Gilbert G. Familial hidradenitis suppurativa: Evidence in favour of single gene transmission. J Med Genet. 1984; 21(4):281-285. The three English families in this report have a total of 21 members (16 females and five males) suffering from chronic hidradenitis suppurativa. The familial nature of HS is occasionally mentioned in published reports but pedigree information is lacking and further detailed studies are necessary if a genetic basis for the disorder is to be confirmed. The wide variation in clinical severity and age at onset of this disease needs to be appreciated. Extensive investigations of two subjects in family B revealed no abnormality of their immunological system. 1974 Familial AC HS Pili Torti and cataracts Gold.pdf Gold SC, Delaney TJ. Familial acne conglobata, hidradenitis suppurativa, pili torti and cataracts. Br J Dermatol. 1974; 91(10):54-7. Male 35 year old, grandfather, mother and sister reported to have same condition. 5 Co-morbid Bone and Joint Disease 2006 HS SAPHO features Ozyemisci-Taskiran.pdf Ozyemisci-Taskiran O, Bolukbasi N, Gogus F. A hidradenitis suppurativa related SAPHO case associated with features resembling spondylarthropathy and proteinuria. Clin Rheumatol. 2006 May; [Epub ahead of print]. We present a 53-year-old man with synovitis-acne-palmoplantar pustulosis-hyperosteosis-osteitis (SAPHO) syndrome who is HLA-B27 positive with a history of uveitis and complicated by proteinuria and osteoporosis. A 53-year-old white male patient presented with a 4-year history of inflammatory back pain which had increased recently. He had troublesome HS for 6 years. Upon failure of medical treatment, surgical excision of the glands in axilla and groin was performed 2 years ago. After a 6-month symptom-free period, pain and foul-smelling discharge recurred for which isotretinoin (0.7 mg kg_1 day_1) was recommended. He had a history of acute anterior uveitis 1 month prior to his admission. No familial history of inflammatory bowel disease or psoriasis was noted. He had no history of oral or genital aphthous ulcerations, erythema nodosum-like lesions, or venous thromboembolism. On physical examination, blood pressure was 160/ 90 mmHg. Dermatologic examination revealed scar formations of previous acneiform lesions on the face and draining nodules, sinus tracts, and postsurgical hypertrophic scars in axillary and groin regions. Thoracic and lumbar spine movements were limited and painful. He was HLA-B27 positive and HLA-B8 negative. Antinuclear antibody (ANA), anti-dsDNA antibody, rheumatoid factor (RF), and Pathergy tests were negative, so were the screenings for viral, parasitic diseases, and brucellosis. Urinalysis revealed moderate proteinuria without red blood cells or casts. In 24-h urine, protein was 637 mg/l with normal creatinine clearance. Renal ultrasonography was unremarkable. Radiographic evaluation of spine showed degenerative changes and osteopenia. Sclerosis and irregularities of sacroiliac joints indicated sacroiliitis radiographically without apparent clinical signs (Fig. 1). Spinal 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 28 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database MRI demonstrated erosive lesions affecting multiple vertebral endplates and fatty infiltration of bone marrow in two vertebral bodies suggested chronic inflammatory process. Bone scan demonstrated increased uptake in the sacroiliac joints, anterior chest wall, and thoracic spine (Fig. 2). Bone mineral density measurements of lumbar and hip regions revealed osteoporosis. Serum calcium, parathyroid hormone, calcitonin, thyroid, prolactine, and sex hormones were normal. 2004 Seronegative arthritis follicular occlusion triad Thein.pdf Thein M, Hogarth MB, Acland K. Seronegative arthritis associated with the follicular occlusion triad. Clin Exp Dermatol. 2004; 29(5):550-552. A 40-year-old Afro-Caribbean man presented with a 1-year-history of tender scalp nodules and pustules, associated with a scarring alopecia Tender nodules and cysts were also noted in his beard area, axillae and groin. At around the same time he developed an asymmetrical arthritis affecting the knees, ankles, elbows, wrists and neck. Possible mechanisms include an inappropriate response to bacterial antigens involved in acne or other autoimmune phenomena. This has analogies to other reactive arthritic syndromes such as SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) and Reiter’s syndrome. SAPHO syndrome is characterized by arthritis and ⁄ or osteitis with preferential anterior chest wall involvement, and although most commonly associated with palmoplantar pustulosis, has been associated with other chronic suppurative skin disorders including hidradenitis suppurativa, acne conglobata, and dissecting cellulitis of the scalp. All patients with acne conglobata, hidradenitis suppurativa, dissecting cellulitis of the scalp, or any combination of these should be asked about musculoskeletal symptoms. 2003 SAPHO Syndrome or concept Earwaker.pdf Earwaker JW, Cotten A. SAPHO: Syndrome or concept? imaging findings. Skeletal Radiol. 2003 Jun; 32(6):311-27. Epub 2003 Apr 29. HS has been described as a feature of this little known skin-bone syndrome. 2000 PAPA SKIN BONE SYNDROME YEON.pdf Yeon HB, Lindor NM, Seidman JG, Seidman CE. Pyogenic arthritis, pyoderma gangrenosum, and acne syndrome maps to chromosome 15q. Am J Hum Genet. 2000 Apr Epub 2000 Mar 21.; 66(4):1443-8. HS has been described as a feature in isolated cases. 1999 Arthritis and HS 48 yo man Hamoir.pdf Hamoir XL, Francois RJ, Van den Haute V, Van Campenhoudt M. Arthritis and hidradenitis suppurativa diagnosed in a 48-year-old man. Skeletal Radiol. 1999; 28(8):453-456. Presentation of one case with HS, acne arthritis, review of literature. 1994 Arthritis associated with HS Bhalla.pdf Bhalla R, Sequeira W. Arthritis associated with hidradenitis suppurativa. Ann Rheum Dis. 1994; 53(1):64-66. Reviews the presentation and clinical findings of arthritis associated with HS. Nine patients identified with HS and arthritis reviewed and compared with previous reports. All nine patients had chronic HS of axilla and groin, three of these also had AC: Axial involvement 4/9, peripheral involvement 9/9 Onset of arthritis occurring 2-15 years after the appearance of skin lesions, activity of the arthritis coincided with skin manifestations in five patients. Two patients had complaints of generalised fatigue, arthralgias and hyperalgesic tender sites indicative of fibromyalgia. One patient had muscle weakness of lower extremity. 2 had conjunctivitis, one of these had keratitis mRH factor negative in 8/9 cases, ANA negative in 7/9 patients. Radiographic abnormalities in all patients, bacterial cultures of HS lesions positive in four out of 9 cases. 1993 Arthropathy HS Rosner.pdf Rosner IA, Burg CG, Wisnieski JJ, Schacter BZ, Richter DE. The clinical spectrum of the arthropathy associated with hidradenitis suppurativa and acne conglobata. J Rheumatol. 1993; 20(4):684-687. Identified a population of patients with HS/AC to estimate prevalence of arthritis in HS/AC, further define its clinical characteristics, and possibly obtain some insight into its pathogenesis. Evaluated all patients seen at university Hospitals of Cleveland with a hospital discharge diagnosis of HS/AC, 44 identified for study, 19 with HS. Out of 21 with signs of arthritis 18 had signs of peripheral arthritis on exam, axial arthropathy present in 15 (low back pain, 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 29 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database stiffness tender sacroiliac joint), involvement of thoracic and cervical spine. Course of arthritis episodic, joint inflammation pattern oligoarticular with 1 to 6 joints involved in individual patients. 15/21 patients with arthritis, skin lesions preceded the onset of arthritis from one to 20 years 3/21 patients skin/bone symptoms began within 6 months of each other 2/21 temporal relationship undefined 4/21 reported worsening of articular symptoms during periods of active skin inflammation TABLE 1 CLINICAL FEATURES OF 44 PATIENTS WITH HS/AC Arthritis No arthritis Arthralgia (N=21) (N=19) (N=4) Age in years mean(range) 38(19-73) 36(10-73) 43(32-52) Sex F:M 11:10 11:8 4:0 Race-Black 17 14 2 -White 3 5 2 -Oriental 1 0 0 HS 19 19 3 AC 9 3 1 Dissect Cell. 4 1 1 Pilonidal C. 3 4 0 Bloods-anemia, leukocytosis, elevated ESR noted, elevated complement levels noted in 12 of 18 patients (67%), none had a positive RF test, 6 had a positive ANA test (30%) HLA frequencies-no significant differences between control population XRAYS-10 of 17 had radiological abnormalities consistent with inflammatory persistant arthritis, 17 of 21 had changes of inflammatory axial arthropathy, changes in the spine seen in 9 patients -all cases with arthritis fall into classification of seronegative spondyloarthropathy _virtually all of the patients had HS as their skin disease and this may signal a predisposition to the development of arthritis 1985 Arthritis and HS Kenik.pdf Kenik J, Hurley J. Arthritis occurring with hidradenitis suppurativa. J Rheumatol. 1985; 12(1):183-184. 27 y.o black male presents with pain, swelling and stiffness of left wrist, PIP and MCP joints of left hand, and arthralgia and stiffness in right wrist. Patienbt had 5 year history of axillary, inguinal and perinal abscess diagnosed as HS. Past medical history of polyarthralgias of hands, wrsts, shoulders and knees without inflammatory signs. On examination patient afebrile despite axillary and inguinal draining lesions with a foul smell. Treated with antibiotics, salicylates, heat pack and skin and joint symptoms responded well. Represented four months later with similar problems. Testing revealed HLA-B27 negative. Symptoms responded to indomethacin. 1984 Arthritis of HS Immunological studies Vasey.pdf Vasey FB, Fenske NA, Clement GB, Bridgeford PH, Germain BF, Espinoza LR. Immunological studies of the arthritis of acne conglobata and hidradenitis suppurativa. Clin Exp Rheumatol. 1984; 2(4):309-311. Purpose-to report six patients between 1978 and 1983 with acne/Hs arthritis in whom transplantation antigens, circulating immune complexes, serum immunoglobulins and complement components were determined -all had antigens to DRw4-circulating immune complexes all raised significantly 6 caucasian patients with acne arthritis 1 with HS (described as chronic suppuration of apocrine glands) 4 with acne conglobata (described as chronic suppuration of eccrine glands) Patient with HS also had PG and symmetrical peripheral arthritis 6 Differential & Missed Diagnosis PERIANAL DISEASE 2005 MRI features of HS Kelly.pdf Kelly A, Cronin P. MRI features of hidradenitis suppurativa and review of the literature . Am J Roent. 2005; 185:12011204. 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 30 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database Case of hidradenitis suppurativa in a 31-year-old woman with Crohn’s disease diagnosed by MRI and confirmed histologically. In our patient, MRI revealed marked thickening of the skin, induration of the subcutaneous tissues, and formation of multiple subcutaneous abscesses. The disease was confined to the skin and subcutaneous tissues of the perineum and medial thighs. No communication with the pelvic organs, such as the bladder, urethra, rectum, or anus, was present. In addition, the lymph nodes in both inguinal regions were enlarged. 2003 Sonography in the diagnosis of HS Kolodziejczak.pdf Kolodziejczak M, Stefanski R, SudolSzopinska I, Jakubowski W. Transrectal and transperineal sonography in the diagnosis of hydradenitis suppurativa. Radiology and Oncology. 2003; 37(3):161-166. The aim of the study was to present the application of transrectal and transperineal sonographies in the differential diagnosis of the hydradenitis suppurativa with the anal fistula. Eight patients (2 women and 6 men, aged between 27-62 years; mean age 54,5 years) with the clinical diagnosis of the anal fistula (6 patients) and the hydradenitis suppurativa (2 patient) were examined. All patients suffered from the recurrent purulent inflammation of the skin and subcutaneous tissue around the anus. 2001 Radiological examination Perirectal Sinus Tracks Fistulas HS Nadgir.pdf Nadgir R, Rubesin SE, Levine MS. Perirectal sinus tracks and fistulas caused by hidradenitis suppurativa. AJR Am J Roentgenol. 2001; 177(2):476-477. A 44-year-old woman presented with abdominal and perineal pain. Radiologists should be aware of the anorectal manifestations of hidradenitis suppurativa because the sinus tracks and fistulas involving the distal rectum can mimic the radiologic appearance of Crohn's disease or actinomycosis infection. 1990 Perianal HS Wiltz.pdf Wiltz O, Schoetz DJ,Jr, Murray JJ, Roberts PL, Coller JA, Veidenheimer MC. Perianal hidradenitis suppurativa. the lahey clinic experience. Dis Colon Rectum. 1990; 33(9):731-734. Retrospective review 43 patients (40 males 3 females) with Perianal HS treated at Lahey Clinic between 1962 and 1987, diagnosis confirmed by pathology, assessment of recurrence and flare-up following surgery based on clinical signs and symptoms at follow-up -median of six years before diagnosis after presenting symptoms of discharge, pain, swelling, pruritis, bleeding and erythema. -35 (81%) had a diagnosis other than HS -70% smokers -all patients were caucasian TABLE 2 INITIAL DIAGNOSIS (N=43) DIAGNOSIS No. OF Patients (PERCENT) Anal fistula 16 37% Pilonidal disease 12 28% Perirectal abcess 7 16% Perianal HS 8 19% TABLE 3 ASSOCIATED CONDITIONS CONDITION No of Persons (percent) Smoking 30 70% Diabetes mellitus 5 12% Obesity 5 12% Crohn's Disease 2 5% TABLE 4 INITIAL SURGICAL PROCEDURES (N=43) PROCEDURE No of patients (percent) Wide local excision 31 72% Incision and drainage 6 14% Local unroofing 4 9% Colostomy/excision 2 5% Table 5 RECURRENCE OR FLARE-UP OF HS (N=29 LOCATION No of Patients (Percent) Recurrence (operative site) 9 31% Flare-up (perianal, not operative site) 12 41% Other sites 8 28% 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 31 Annotations MSAB Database Table 6 NUMBER OF RECURRENCES AND FLAREUPS IN PERIANAL AREA REQUIRING FURTHER TREATMENT (N=21) NUMBER Number of persons (Per cent) 1 10 48% 2 3 14% 3 5 24% >3 3 14% TABLE 8 INITIAL SURGICAL TREATMENT IN PATIENTS WITHOUT RECURRENCE (N=14) TREATMENT No of patients (percent) Wide local excision 11/14 79% Incision and drainage/unroofing 3/14 21% __________________________________________________ BREAST/AXILLA 2005 Case study Galactoceles and HS Imaging Dx Rampaul.pdf Rampaul RS, Chakrabarti J, Burrell H, Evans AJ, Macmillan D. A tail of two axillae. Breast J. 2005 Mar-Apr; 11(2):160-1. A 31-year-old woman presented with a 1-week history of bilateral swellings of the axillae, having been diagnosed with bilateral hidradenitis suppurativa 4 years earlier. At this presentation she was 3 weeks postpartum. Examination revealed engorged lactating breasts and both axillae were swollen with numerous discrete palpable lumps each measuring about 1.5 cm within each axilla. There was no discharge from the axillae. Ultrasonography revealed multiple thin-walled cysts, but with no fluid levels that were consistent with bilateral galactoceles (Fig. 1), and a cyst in the upper outer quadrant of the left breast which, on aspiration was shown to contain milk. The axillary cysts were not aspirated, as they were identical on imaging to the breast galactocele. 2004 MRI accessory breast tissue may be misdiagnosed as HS Laor.pdf Laor T, Collins MH, Emery KH, Donnelly LF, Bove KE, Ballard ET. MRI appearance of accessory breast tissue: A diagnostic consideration for anaxillary mass in a peripubertal or pubertal girl. AJR Am J Roentgenol. 2004 Dec; 183(6):1779-81. Diagnostic consideration for axillary mass. 2003 Axillary breast tissue mistaken for HS Silverberg.pdf Silverberg MA, Rahman MZ. Axillary breast tissue mistaken for suppurative hidradenitis: An avoidable error. J Emerg Med. 2003; 25(1):51-55. The patient is a 28-year-old African-American woman who presented to the Emergency Department (ED) approximately14 days after she noticed a mass in her left axilla. 2002 Mammillary fistula as a manifestation of HS Cosman.pdf Cosman BC, Al-Refaie WB. Mammillary fistula as a manifestation of acne inversa (hidradenitis suppurativa): Report of two cases. J Am Coll Surg. 2002; 194(6):829-833. We present two patients with MF and concurrent acne inversa/HS lesions. These cases raise the question of whether MF might be a manifestation of acne inversa. Despite the ample literature on MF and the very extensive literature on HS, this is a previously unrecognized association, suggesting either that these cases are unusual or that they demonstrate something that has not been seen because it has not been looked for. In its lack of description of associated conditions, the literature contains the implicit assumption that MF is an isolated lesion of the breast. Our cases challenge that assumption, and it remains for surgeons who see MF frequently to confirm or deny the suggested association with acne inversa/HS. OTHER 2006 Pachyonychia Congenita diff dx HS GJB2 mut. Smith.pdf 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 32 Annotations MSAB Database Smith F, Kaspar R, Schwartz M, McLean W, Leachman S. Pachyonychia Congenita. In: GeneReviews at GeneTests: Medical Genetics Information Resource (database online).<http://www.genetests.org>. Accessed 2/22/06. University of Washington, Seattle, WA, United States., 2006 Jan. HS and Follicular Occlusion Triad caused by mutations of the gene GJB6 (Connexin 30) could possibly be confused with PC. 2005 Pachyonychia Congenita may resemble HS Leachman.pdf Leachman S, Kaspar R, Fleckman P, et al. Clinical and pathological features of pachyonychia congenita. J Investig Dermatol Symp Proc. 2005 Oct; 10(1):3-17. A form of Pachyonchyia Congenita (PC) may resemble HS. 2005 ABSTRACT Axillary chloracne may mimic HS Holstege.pdf Holstege CP. Effects of Dioxins on Human Health Clinical Toxicology, 43:21-22, 2005 Abstracts of the European Association of Poisons Centres and Clinical Toxicologists XXV International Congress <http://www.eapcct.org/publicfile.php?folder=congress&file=Abstracts_Berlin.pdf>. Accessed10/30/2005. EAPCTT, Berlin, Germany, 2005. Lesions in the axilla may mimic hidradenitis suppurativa. 2004 ABSTRACT Multisystem Langerhans Cell Histiocytosis HS Sheu.pdf Sheu MY, Morrison L, White C. A 20 year history of untreated multisystem langerhans cell histiocytosis• ABSTRACT. J Am Acad Dermatol. 2004 March; 50(3(Supp. 1)):46. Initial diagnosis with HS. 2000 Tuberculous anal sepsis misdiagnosed as HS Kraemer.pdf Kraemer, M.;Gill, SS.;Seow-Choen, F. Tuberculous anal sepsis: report of clinical features in 20 cases. Dis Colon Rectum. 2000 Nov;43(11):1589-91. TWO PATIENTS MISDIAGNOSIS WITH HS Two patients with tuberculous abscesses without identifiable fistulas healed after drainage procedure and anti-tubercular chemptherapy. These two were misdiagnosed as Hidradenitis Suppurativa initially. 1996 ANCA disease possible misdiagnosis as HS Irvine.pdf Irvine, AD.;Bruce, IN.;Walsh, M.;Burrows, D.;Handley, J. Dermatological presentation of disease associated with antineutrophil cytoplasmic antibodies: a report of two contrasting cases and a review of the literature. Br J Dermatol. 1996 May;134(5):924-8. PERHAPS UNCLEAR BUT_ANCA ASSOCIATED DISEASE MISDIAGNOSED AS HIDRADENITIS SUPPURATIVA LEADING TO DEATH 31 DAYS AFTER SURGERY FOR EXCISION AND SKIN GRAFTING OF BILATERAL INGUINAL HS 7 Immunity & Inflammation BACTERIA / ANTIBIOTICS 2005 ABSTRACT Combination therapy Clindamycin rifampicin HS Mendonca.pdf Mendonca C, Griffiths CEM. Combination therapy with clindamycin and rifampicin is effective for hidradenitis suppurativa • ABSTRACT. J Am Acad Dermatol. 2005 March; 52(3 Supplement 1):P47. We report our experience of the use of rifampicin 300 mg and clindamycin 300 mg both twice daily for 10 weeks in the treatment of HS. Fourteen patients were treated with this combination of antibiotics between 1998 and 2003 (9 women, 5 men). Eight patients (6 female, 4 male) achieved complete remission of HS between 1 and 4 years after only one course of rifampicin and clindamycin treatment, and another two patients achieved remission after substituting minocycline (100 mg) for clindamycin because of transient diarrhea. Subsequently, these 10 patients have not relapsed. Seven responders had only perineal involvement; 1 had perineal,axillary, and breast involvement; and 2 had perineal and axillary involvement only. Four patients were unable to complete the course because of diarrhea. 1999 Microbiology of HS Brook.pdf 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 33 Annotations MSAB Database Brook I, Frazier EH. Aerobic and anaerobic microbiology of axillary hidradenitis suppurativa. J Med Microbiol. 1999; 48(1):103-105. A retrospective review of the microbiological and clinical data of 17 specimens obtained from axillary hidradenitis suppurativa (HS) over a period of 6 years was undertaken to study the aerobic and anaerobic microbiology of this condition. A total of 42 bacterial isolates (2.5 per specimen) were obtained, 12 aerobic or facultative (0.7 per specimen) and 30 anaerobic or micro-aerophilic (1.8 per specimen). Aerobic and facultative bacteria only were isolated in six (35%) cases, anaerobic bacteria only in seven (41%) and mixed aerobic and anaerobic bacteria in four (24%). The predominant aerobic bacteria were Staphylococcus aureus (six isolates), Streptococcus pyogenes (three) and Pseudomonas aeruginosa (two). The most frequently isolated anaerobes were Peptostreptococcus spp. (10), Prevotella spp. (seven), micro-aerophilic streptococci (four), Fusobacterium spp. (three) and Bacteroides spp. sensu stricto (three). This study highlights the polymicrobial nature and predominance of anaerobic bacteria in axillary HS and the need for antimicrobial thereby to reflect this. BACTERIA isolated from 17 HS specimens AEROBIC BACTERIA ANAEROBIC AND MICRO-AEROPHILIC BACTERIA S. Aureus 6 Pstr. magnus 1 S. epidermis 1 Pstr. prevotii 3 Str. pyogenes 3 Pstr. asaccharolyticus 2 P. aeruginosa 2 Peptostreptoccus spp 4 Gemella morbilliorum 2 Str. Intermidius 1 Micro-aerophilic strept. 1 F. nucleatum 2 F. varium 1 B. fragilis 2 B. ovatus 1 B. urealyticus 1 Pr. oris-buccae 2 Pr. melaninogenica 3 Pr. intermedia 2 Porphyromonas asacch. 2 TOTAL 12 30 1998 Topical clindamycin for HS Jemec.pdf Jemec GB. Topical clindamycin versus systemic tetracycline in the treatment of hidradenitis suppurativa. J Am Acad Dermatol. 1998; 39(6):971. We have conducted an RCT based on the previously published trial(Clemmenson), and compared the clinical efficacy of topical clindamycin against that of systemic tetracycline focusing on the disease assessment in an attempt to provide a better criteria for future trials in hidradenitis. No significant differences were found between the two treatments. The presence of bacteria at the onset of treatment did not affect the outcome. Patients assessed the disease as significantly worse than physicians in 3 of 5 evaluations (P = .0096 to .015). Patient soreness did not appear to change during the course of the study. The most important factor in the patients’ overall assessment of disease severity appears to be soreness. In lieu of true objective measures of disease activity in hidradenitis suppurativa it is, therefore, suggested that standard clinical outcome variables are supplemented with records of the patients’ subjective assessment of pain and overall disease activity. 1983 Topical Rx HS with Clindamycin Clemmenson.pdf Clemmensen OJ. Topical treatment of hidradenitis suppurativa with clindamycin. Int J Dermatol. 1983; 22(5):325-328. Due to the possible pathogenic relation between acne and HS and the known beneficial effect of topical clindamycin on acne, we determined to evaluate the effect of this drug locally on HS. 30 consecutive patients with HS of the axillae and/or groins were included in the study. 27 concluded the trial, 21 women and six men. Mean age 31.3 years (range 18-59 years), Duration of HS ranged between one and ten years (mean 5.5 years) and was not correlated to the intensity of the disease. Cyclic recurrence of HS perimenstrual in 9/21 females. 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 34 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database Inclusion criteria: symptoms of HS for more than six months and with intervals of recurrence shorter than six weeks. Exclusion criteria- pregnant women and patients who had received either systemic or local antibiotic treatment within the last four weeks. 12 week double blind trial of either clindamycin hydrochloride 1% in a vehicle of isopropanol 80%, propyleneglycol 10%, and water 9% or placebo (vehicle alone), review every four weeks, number of putules, inflammatory nodules and abscesses counted.-comedones were often found. Since they were not regarded as criteria for acceptance in the study they were not counted 13 received active treatment, 14 received placebo In comparison to placebo treatment with clindamycin led to improvements in patient assessment and improvements in lesion parameters/scores, in comparison to the controls on placebo only. Some of those on placebo were noted to have improvement in patient self-assessment and lesion parameters. ISOTRETINOIN 1999 Letter to editor Isotretinoin for HS Jemec.pdf Jemec GB. Long-term results of isotretinoin in the treatment of 68 patients with hidradenitis suppurativa. J Am Acad Dermatol. 1999; 41(4):658. Opinion: "Despite the follicular nature of both diseases, the analogy between hidradenitis suppurativa and acne is limited, at least in therapy, as the present study has shown. In acne therapy isotretinoin is established as a reference drug, whereas it is obviously not in hidradenitis." 1999 Long-term results isotretinoin for HS Boer.pdf Boer J, van Gemert MJ. Long-term results of isotretinoin in the treatment of 68 patients with hidradenitis suppurativa. J Am Acad Dermatol. 1999; 40(1):73-76. In an initial report we described our results from administration of low-dose isotretinoin to 21 patients with HS; patients were observed for up to 24 months. In the present study we enrolled 68 patients, including the already mentioned 21 patients, and focused on the response rate, longterm follow-up, and the possibility of a relation between response rate and severity. 68 patients studied, 59 females, 9 males. 8 patients had HS and acne conglobata. Affected areas: Genitocrural-45, Axillary-6, Axillary and genitocrural-17 48 patients completed treatment treated for 4-6 months. Follow-up at 6 to 107 months(9 years) (mean 46 months). 16 (23.5%) achieved a score of +3 (clear) at the end of treatment. 14 (20.68%) achieved a score of +2 (marked improvement). 11 (16.2%) achieved a score of +1 (improvement). 7 (10.3%) achieved a score of 0 (no change). 20 (29.4%) dropped out of study. No control group utilized. Conclusion-monotherapy of Hs with isotretinoin is of limited value for HS. 1998 Isotretinoin treatment failures HS Leyden.pdf Leyden J. The role of isotretinoin in the treatment of acne: Personal observations . J Am Acad Dermatol. 1998 Aug; 39(2):45-49. _______________________________________________________ Table III. Patients with a high probability of relapse or failure with isotretinoin Preteens and young teenagers Sinus tract lesions Excess granulation tissue Androgen excess ________________________________________________________ By far the most common cause of failure to respond to therapy is the poor response of epithelial sinus tracts. Patients with a personal or family history of sinus tract disease (such as pilonidal sinus, hidradenitis, or dissecting cellulitis of the scalp) are prone to form undermining epithelial sinus tracts when sebaceous follicles are disrupted. Identification of these lesions before isotretinoin therapy can help prepare a patient for the potential need for surgical removal if intralesional steroids and isotretinoin are not successful. 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 35 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database 1986 Familial Widespread HS Treatment Failure Isotretinoin Norris_Cunliffe.pdf Norris JF, Cunliffe WJ. Failure of treatment of familial widespread hidradenitis suppurativa with isotretinoin. Clin Exp Dermatol. 1986; 11(6):579-583. Isotretinoin has been used in the treatment of HS with varying degrees of success. We investigated a family of six subjects(2 sisters and their four children mentioned in Fitzsimmons 1984 family study) with widespread HS and treated them with isotretinoin at a dose of 1mg/kg for 4 months. No improvement was obtained and the subjects noted a flare in their disease activity (severe in two patients) after stopping the drug. The rationale for treating HS with isotretinoin is called into question. IMPORTANT EVIDENCE and CITATIONS USED BY AUTHORS 1."In the initial trials of isotretinoin in acne, (the authors) included a single patient with severe HS who falied to respond after a four month course on an unspecified dose between 0.1 and 1.0 mg/kg/day" Jones,DH.;Blanc,D.;Cunliffe,WJ. 13-cis-retinoic acid and acne. Lancet 1980,ii, 1048-1049 2. Authors "reported three patients with long-standing HS who were treated with 1.0 mg/kg/day isotretinoin, or less for 16 weeks, no improvement was forthcoming. It was suggested that higher doses might be needed, or that the drug should be given at an earlier stage before the effects of scarring had taken their toll." Jones DH, Cunliffe WJ, King K. Hidradenitis suppurativa-lack of success with 13-cis-retinoic acid. Br J Dermatol. 1982 Aug;107(2):252. 3. Authors "make an important comparison between HS and severe acne which is resistant to isotretinoin-both conditions are characterized by multiple, dissecting sinus tract type of lesions which do poorly with isotretinoin montherapy but repond better to combination treatment with intralesional steroids, systemic antibiotics and local surgery, in addition to isotretinoin. Shalita AR, Cunningham WJ, Leyden JJ, Pochi PE, Strauss JS. Isotretinoin treatment of acne and related disorders: an update. J Am Acad Dermatol. 1983 Oct;9(4):629-38. Patients 2 sisters, aged 45 and 47, four children ages ranged from 14 to 28 year olds. All had HS affecting the axillae, groins, perineum and natal cleft and which, although beginning between the ages of four and five, did not become a serious problem until adolescence. The lesions consisted of both inflamed and quiescent nodules, bridged and atrophic scars and multiple, frequently double comedones. These same changes were also found on the buttocks, thighs, midriff and upper back of all except the youngest patient. Two patients had lesions around the neck and two of the females had submammary lesions. An unusual feature was that of discrete areas of disease activity that affected the antecubital fossae in two patients and the popliteal fossae of a third. The most striking finding in this study, which has not been noted before, was the very troublesome rebound effect in disease activity that all the patients noted when isotretinoin was stopped. This might suggest perhaps that there had been subtle and unnoticed improvement in the HS during treatment, but neither the patients nor ourselves thought that this was the case. The reason for this rebound is not immediately apparent, but it may perhaps have a parallel in the flare of acne that can occur during early isotretinoin treatment. At first sight it appears not unreasonable to use isotretinoin for HS because HS and acne do occur in the same patient and isotretinoin is known to be a very effective treatment for acne, It is however, a matter of simple clinical observation that the majority of patients with acne do not have HS, and that patients with HS do not invariably have acne. Any therapeutic parallels, therefore, that we may wish to draw between HS and acne should recognise that these disorders, are dissociated more often than they are associated, and thus any treatment modality that is effective in one need not necessarily be effective in the other. 1982 Lack of success with 13-cis-retinoic acid for HS Jones_Cunliffe.pdf Jones DH, Cunliffe WJ, King K. Hidradenitis suppurativa-lack of success with 13-cis-retinoic acid. Br J Dermatol. 1982; 107(2):252. Three patients not responsive to isotretinoin therapy for HS. IMMUNE MODULATION 2006 Poster abstract_Intramuscular Ig for HS Goo.pdf 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 36 Annotations MSAB Database Goo B, Chung K, Chung W. P528 intramuscular immunoglobulin for recalcitrant suppurative skin diseases. poster abstracts, american academy of dermatology, 64th annual meeting, march 3–7, 2006 san francisco, CA. J Am Acad Dermatol. 2006 Mar; 54(3):AB58. IM Ig as a treatment for suppurative disease. 2005 POSTER HS treated with Dapsone Gupta.pdf Gupta S, Taibjee S, Lewis H. Hidradenitis suppurativa treated with dapsone, a case series of six patients, BRISTOL CUP POSTER. Br J Dermatol. 2005 June; 153(S1):20-51. Six cases: 3 female,1 male( treated with dapsone. Age range 31–51 years) with hidradenitis suppurativa (mean duration 9·2 years), treated with dapsone between 2002 and 2004. Dapsone 50 mg daily was commenced in all patients. One patient responded at this dose, three patients required 100 mg daily and one patient 150 mg daily. These five patients reported a significant improvement in disease activity, with a reduction in the frequency, and severity of lesions as well as symptomatic improvement.Time to response varied between 4 and 12 weeks. Treatment was well-tolerated in all patients with no significant adverse effects. All five patients continue on dapsone 50–50mg daily and are unable to reduce or stop dapsone due to relapse of their disease. The mean duration of treatment is 15·6 months (range 6–31). One patient was lost to follow-up while on dapsone 100 mg daily. 2005 Rx HS oral ciclosporin Rose.pdf Rose R, Goodfield M, Clark S. Treatment of recalcitrant hidradenitis suppurativa with oral ciclosporin. Clin Exp Dermatol Online Early. 2005; 0(0) Two cases (1 male,1 female) HS treated with oral ciclosporin. 2002 Methotrexate Limited value in Rx HS Jemec.pdf Jemec GB. Methotrexate is of limited value in the treatment of hidradenitis suppurativa. Clin Exp Dermatol. 2002; 27(6):528-529. Based on the morphological and histological similarities, as well as the possible co-occurrence of HS and CD it was therefore speculated that therapies used for CD might also be effective in HS. Three patients suffering from therapy resistant HS were treated with oral MTX in an open study. the primary lesions of the disease appeared unaffected by treatment with MTX, and the frequency of flare-ups was unchanged. ANTI-TNFA TREATMENT 2006 Poster abstract_Infliximab for HS Armario-Hita.pdf Armario-Hita J, Fernandez-Vozmediano J. P525 infliximab for the treatment of suppurative chronic hidradenitis. poster abstracts, american academy of dermatology, 64th annual meeting, march 3–7, 2006 san francisco, CA. J Am Acad Dermatol. 2006 Mar; 54(3):AB57. 6 patients treated 2006 Poster abstract_Efalizumab for HS Robins.pdf Robins D. P594 successful treatment of hidradenitis suppurativa with efalizumab: A case report. poster abstracts, american academy of dermatology, 64th annual meeting, march 3–7, 2006 san francisco, CA. J Am Acad Dermatol. 2006 Mar; 54(3):AB74 Case Report 2006 Etanercept HS Cusack.pdf Cusack C, Buckley C. Etanercept: Effective in the management of hidradenitis suppurativa. Br J Dermatol. 2006 Apr; 154(4):726-9. Objectives: TNF-a is implicated in many inflammatory disorders and we wished to determine the efficacy of subcutaneous etanercept, a competitive inhibitor of TNF-a in the control of HS symptoms. Inclusion/Exclusion criteria: severe (Hurley stage II and above)7 chronic HS unresponsive to at least two conventional treatments. Previous historiesof tuberculosis or malignancy were exclusion criteria. Patients: mean age of 32.3 (range 16–42) years. The average disease duration was 5.7 (range 4–11) years and all patients had multifocal involvement. Previous therapies included high dose oral antibiotics (flucloxacillin, oxytetracycline, minocycline), dapsone, isotretinoin, rifampicin, 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 37 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database tacrolimus, antiandrogenic oral contraceptives and radical surgery. Patient 2, who had a 13-year history of severe Crohn’s disease, presented with a 4-year history of recurrent abscesses in her axillae, groins, perineum and inner thighs. Biopsy of a groin abscess at another hospital showed histological changes consistent with HS. Methods: December 2003, we commenced six FEMALE patients with severe, recalcitrant HS on etanercept (25 mg subcutaneously twice weekly in all cases). Patients self-assessed their disease activity and completed Dermatology Life Quality Index (DLQI) questionnaires immediately before the introduction of therapy and 24 weeks later in the case of four patients, and 12 weeks later in the case of two others. All patients were asked to estimate the time lapse between commencement of treatment and initial response. Patient 6 has Downs Syndrome DLQI NOT self-assessed, DLQI and reduction of disease activity estimated by carer. Results: Treatment was well tolerated by all patients with no reported adverse reactions: (Except for an increased incidence of mild self-resolving URTI's in one patient. A marked reduction in selfreported disease activity (mean reduction of 61% at 24 weeks), in DLQI scores (mean reduction of 64% at 24 weeks) and in relapse rates occurred. All patients rated etanercept as their most effective treatment to date. On examination, patients had markedly reduced tenderness, induration and sinus drainage. 4/6 patients were administered antibiotics for relapses of HS whilst being treated with etanercept. Conclusions: Our results show the effectiveness of etanercept in this group of female patients with particularly challenging disease. Etanercept, unlike infliximab, may be administered subcutaneously, rendering costly day-case admissions unnecessary. 2/6 patients had relapses of HS upon discontinuing treatment, Patient 3 discontinued etanercept in anticipation of pregnancy, another patient discontinued treatment in error. 2005 Treatment of HS with Infliximab in a patient with Crohn's Rosi Rosi YL, Lowe L, Kang S. Treatment of hidradenitis suppurativa with infliximab in a patient with crohn's disease. J Dermatol Treat. 2005; 16(1):58-62. This case study proposes a novel theory of HS pathogenesis called 'autoinflammation'. This case involves a patient with inactive Crohn's disease who had a diagnosis of HS confirmed with histopathology, despite being assessed for differential diagnoses of cutaneous Crohn's disease and bacterial infection. Treatment of the HS with an anti-TNFa medication led to an immediately positive effect. 2005 ABSTRACT HS treated with adalimumab AAD Ravat.pdf Ravat F, O'Reilly D, Handfield-Jones S. Recalcitrant hidradenitis suppurativa treated with adalimuab . J Am Acad Dermatol. 2005 March; 52(3 Supplement 1):P58. Case study 1 male treated with infliximab, relapsed HS after 9 treatments placed on adalimumab. Our case demonstrates that although infliximab has been effective for our patient in the short term, its effectiveness has not been sustained. 2004 POSTER TNFa Blockers for HS Massarotti.pdf Massarotti E, Sobell J. TNF-alpha blockers for hidradenitis suppurativa American College of Rheumatology 2004 meeting, October 16-21, 2004, San Antonio, TX <http://www.abstractsonline.com>. Accessed 6/12/2005. American College Rheumatology, United States, 2004. 2 male cases HS treated with Infliximab 2004 ABSTRACT HS in Crohn's treated with Infliximab Stipho.pdf Stipho S, Welch R, Feller E, Shah S. Hidradenitis suppurativa in Crohn’s disease responding to infliximab. abstracts submitted for the 69th annual scientific meeting of the american college of gastroenterology. American Journal of Gastroenterology. 2004 Oct; 99(S10):S163. We present a patient with Crohn’s disease who developed refractory HS, which responded to Infliximab therapy. Case Report: A 34 year old female was diagnosed with Crohn’s disease at age 12 following recurrent episodes of crampy abdominal pain and diarrhea. Her disease was refractory to medical therapy, resulting in eventual colectomy and ileostomy at age 20. The patient subsequently remained well with occasional bouts of abdominal discomfort and increased ileostomy output. She later presented with an 8 month history of progressive lesions involving genital, inguinal, pubic and perianal areas. These lesions were erythematous, painful, keloid-like plaques with nodules and draining sinuses. The patient recalled similar skin lesions flaring with each pregnancy. These lesions had previously responded inconsistently to topical and oral antibiotics. A skin biopsy indicated hidradenitis suppurativa. PAS, AFB and GMS staining were all negative for microorganisms. Gram staining and culture were positive for sparse growth of 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 38 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database staph. aureus and beta hemolytic strep. After a course of cephalexin, the lesions remained unchanged.Apelvic MRI showed no fistula or abscess. A trial of topical acetic acid and ketoconazole cream and oral doxycycline was given with no response. A literature search found four case reports and one small case series of HS responding to infliximab. Therapy was subsequently initiated with infliximab at a dose of 5mg/kg, given at 0, 2 and 6 weeks. The patient had a rapid response with significant alleviation of symptoms and resolution of lesions. Current medical therapies are inadequate in treating HS. Infliximab shows promise in the treatment of severe HS. Further studies addressing the efficacy and safety of infliximab and its effect on HS disease course are needed. The dramatic response to infliximab in our patient and in previous cases suggests a role for TNF in the pathogenesis of HS. 2003 HS Infliximab Lebwohl.pdf Lebwohl B, Sapadin AN. Infliximab for the treatment of hidradenitis suppurativa. Journal- American Academy of Dermatology. 2003; 49(5):275-276. 1 male patient initially misdiagnosed with Crohn's Disease which was then subsequently diagnosed as HS and then treated with Infliximab. 2003 HS Infliximab Adams.pdf Adams DR, Gordon KB, Devenyi AG, Ioffreda MD. Severe hidradenitis suppurativa treated with infliximab infusion. Arch Dermatol. 2003; 139(12):1540-1542. 1 male case of HS treated with Infliximab. 2003 HS Infliximab Sullivan Kirsner.pdf Sullivan TP, Welsh E, Kerdel FA, Burdick AE, Kirsner RS. Infliximab for hidradenitis suppurativa. Br J Dermatol. 2003; 149(5):1046-1049. Retrospective review of five patients (4 female, 1 male) treated with infliximab for HS. Three had marked improvement, two had moderate improvement. While receiving Infliximab, two patients continued on prednisone, and one on ciclosporine. All patients reported that Infliximab had been their most effective therapy to date. 1 out of 5 treated for presumed Mycobacterium tuberculosis related (assumed) submandibular swelling. "This series is limited by its retrospective nature, its lack of a control group and the fact that neither patients nor physicians were blinded. Despite the potential for bias, we feel that our clinical success warrants further evaluation of infliximab for this difficult-to-treat condition. In this series both physicians and patients reported rapid improvement and a diminution in the extent of HS after infliximab therapy." 2003 HS Crohn's TNF Infliximab Roussomoustakaki.pdf Roussomoustakaki M, Dimoulios P, Chatzicostas C, et al. Hidradenitis suppurativa associated with crohn's disease and spondyloarthropathy: Response to anti-TNF therapy. J Gastroenterol. 2003; 38(10):1000-1004. Case Study 2001 HS Crohn's disease treated with Infliximab Martinez.pdf Martinez F, Nos P, Benlloch S, Ponce J. Hidradenitis suppurativa and crohn's disease: Response to treatment with infliximab. Inflamm Bowel Dis. 2001; 7(4):323-326. 30 year old woman developed perianal lesions in Feb 1999 and axillary lesions Dec 1999 and was diagnosed with HS (six years after Crohn's Disease diagnosis). In our patient, therapy with anti-TNF was initiated because of the lack of response to treatment with antibiotics (metronidazole and ciprofloxacin) and surgical drainage. Both perianal and axillary lesions recovered quickly after anti-TNF administration, remaining inactive with maintaining treatment with azathioprine. 8 Hormones 2005 HS Finasteride Joseph.pdf Joseph M, Jayaseelan E, Ganapathi B, Stephen J. Hidradenitis suppurativa treated with finasteride. J Dermatolog Treat. 2005 Apr; 16(2):75-8. 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 39 Annotations MSAB Database Five women, two men treated with finasteride (selective inhibitor of Type 11 5a reductase), six improved. two patients had recurrences 1 mth after ceasing treatment. 1 patient referred to surgery. 1 patient discontinued drug due to pruritis. Enlargement of breasts in two women, in one persisting almost one year. 2003 HS and myotonic dystrophy Cooper Dawber.pdf Cooper SM, Dawber RP, Hilton-Jones D. Three cases of androgen-dependent disease associated with myotonic dystrophy. J Eur Acad Dermatol Venereol. 2003; 17(1):56-58. Our three cases all demonstrate androgen-dependent diseases: male-pattern alopecia, hidradenitis suppurativa, keratosis pilaris and acne. 1999 Two cases Prepubertal HS Mengesha.pdf Mengesha YM, Holcombe TC, Hansen RC. Prepubertal hidradenitis suppurativa: Two case reports and review of the literature. Pediatr Dermatol. 1999; 16(4):292-296. Onset of HS predates puberty 1999 Finasteride as a therapy for HS Farrell.pdf Farrell AM, Randall VA, Vafaee T, Dawber RP. Finasteride as a therapy for hidradenitis suppurativa. Br J Dermatol. 1999; 141(6):1138-1139. These (two) initial cases suggest that finasteride may be beneficial in hidradenitis suppurativa, and in view of the previous persistent and very recalcitrant nature of our patients' symptoms and signs, we are currently extending this study and examining biopsies of involved and uninvolved sites of more patients with hidradenitis suppurativa to determine whether expression of 5-a reductase type I or type II is occurring in this condition. 1997 Sebum excretion HS Jemec.pdf Jemec GB, Gniadecka M. Sebum excretion in hidradenitis suppurativa. Dermatology. 1997; 194(4):325-328. Study to investigate sebum excretion and markers of cutaneous virilization in HS. 16 women with HS 16 female controls, matched for age and sex and assessed during last two weeks of menstrual cycle. 6 cases and seven controls were using OCP (none containing cyproterone) at time of exam SEBUM-cheeks,axillae,genitofemoral folds cleaned, shaved, sebutape applied for 1 hour no significant difference to controls, pattern of SE and density of active glands similar -does not exclude an overlapping pathogenesis of HS with acne (mechanical acne and chloracne may occur without seborrhea ANDROGEN EXCESS-following biological markers of androgen excess were studied: Androgenic alopecia, hirsutism, HS, and acne vulgaris scores and BMI measured. no significant difference to controls (Note-Obese HS:Control 6:1) In this study the standardized clinical assessment of cutaneous virilization found no significant difference between patients and healthy controls although it should be noted that the variation ijn hirsutism and acne scores in HS patients is much larger than in controls. 1996 Endocrine factors pre and post menapausal women Barth.pdf Barth JH, Layton AM, Cunliffe WJ. Endocrine factors in pre- and postmenopausal women with hidradenitis suppurativa. Br J Dermatol. 1996; 134(6):1057-1059. Review of 66 women with HS in order to re-examine the endocrinology of HS. We have not been able to show an abnormalincrease in androgens, or suppression of SHBG, in females with HS, compared with controls matched for hirsutism. _________________________________________________________________________________ TABLE 1 Plasma androgens in women with HS show no significant differences from women controlled for weight and hirsuties (values Quote as median (range): mann-Whitney U test. Hirsuties is defined as a Ferriman and Gallwey score of>10 TESTOS. SHBG DHAS HS without hirsuties(n=18) 1.6(0.7-2.1) 44.5(17-103) 5.4(1.7-14.1) Weight matched 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 40 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database non-hirsute controls (n=27) 1.5(0.8-2.7) 45(12-78) 4.3(2.3-13.6) HS with hirsuties (n=16) 1.6(1.2-2.5) 27(11-69) 6(1.6-13.9) Weight matched hirsute controls (n=23) 2.0(0.9-4.2) 30(7-53) 7.1(0.9-14.9) ________________________________________________________________________________________ 1991 Androgen metabolism by isolated apocrine glands Barth.pdf Barth JH, Kealey T. Androgen metabolism by isolated human axillary apocrine glands in hidradenitis suppurativa. Br J Dermatol. 1991; 125(4):304-308. Androgen metabolism was investigated in normal human apocrine glands and in those isolated from age-matched patients with hidradenitis suppurativa. Axillary skin was obtained under LA from five female patients with HS (median age 35 years, range 25-44 years). All suffered from axillary disease, the skin was not sampled from an inflamed or scarred site. Control axillary skin was obtained from 5 women of simialr age undergoing staging for breast cancer (median age 41 years, range 38-46 years). None of the control women had any features of androgen excess. Axillary glands were isolated by shearing and androgen interconverting enzyme activities were measured in cell-free homogenates by incubation with [3H] dehydroepiandrosterone, [3H] androstenedione and [3H] testosterone. The activities (pmol/mg protein/min: mean + SEM) of 3 beta-hydroxysteroid dehydrogenase delta 4-5-isomerase (10.0 +/1.2 vs. 5.3 +/- 0.5: n = 5) and 17 beta-hydroxysteroid dehydrogenase (58.1 +/- 4.5 vs. 35.7 +/- 5.2: n = 5) were significantly lower (P less than 0.005) in hidradenitis suppurativa, whereas 5 alpha-reductase activity (12.5 +/- 2.3 vs. 12.5 +/- 2.0: n = 5) was similar. This report suggests that hidradenitis suppurativa cannot be attributed to exaggerated activities of end-organ androgen interconverting enzymes. The finding that the enzyme activities were not raised is perhaps not surprising as the apocrine gland is not enlarged in HS (Morgan and Hughes 1979 ). MAKES COMPARISON TO ACNE that in acne, raised enzyme activities are due to hypertrophy of sebaceous glands and hair follicles HSF SUGGESTION/QUERY: What are the demonstrable enzyme activities in unaffected hair follicles of HS patients which may be enlarged/hypertrophic? 1989 Lipogenesis isolated apocrine glands testosterone lack of maintenance effect Barth.pdf Barth J, Ridden J, Philpott M, Greenall M, Kealey T. Lipogenesis by isolated human apocrine sweat glands: Testosterone has no effect during long-term organ maintenance. J Invest Dermatol. 1989 Mar; 92(3):333-6. Describes the nature/composition of apocrine secretion. Glands maintained in vitro with and without testosterone, no differences with these glands noted with or without testosterone. 1989 Isolated apocrine glands as model for HS Barth.pdf Barth J, Kealey T. Isolated human apocrine sweat glands as a model for hidradenitis suppurativa. In: Marks R, Plewig G (eds). Acne and Related Disorders. London,UK: Martin Dunitz, 1989:371-374. Systematic analysis of lipid precursors of apocrine secretions produced by isolated apocrine glands, proposed as a means and method to study pathogenesis of HS. 1989 Characterization of endocrine lesion HS Harrison.pdf Harrison B, Hughes L. Characterization of the endocrine 'lesion' in hidradenitis suppurativa. In: Marks R, Plewig G (eds). Acne and Related Disorders. London,UK.: Martin Dunitz, 1989:361–363. Specific clinical and biochemical features have been examined in a large series of patients to assess further the relationship between the clinical presentation and the putative underlying androgen disorder. 152 patients with hidradenitis were referred to the University Department of Surgery in Cardiff between 1978 and 1986. 109 women (71.7%) 43 men (28.3%) Mean age at onset for men was 24.5+/- 9 years (range ,1-35 years, mean age at onset for women was 24.9 +/- 10 years (1-51 years. GYNAECOLOGICAL STATUS(EXCLUDING MEN): Regular cycles, 25-31 days, were described by 56/109 women(8 of these on OCP), irregular cycles described by 25/109. Note-28 WOMEN MAY HAVE NO RESPONSES RECORDED? 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 41 Annotations MSAB Database Note-According to table 47/83 responses recorded to premenstrual flare (57%) or expressed in relation to total women 47/109 (43%) 26 WOMEN MAY HAVE NO RECORDED RESPONSES BODY WEIGHT/BMI (INCLUDING MEN): Table provided may not be accurate 31 men (77% overweight + 26% obese=103% overweight and obese) Table provided may not be accurate 78 women (69% overweight + 33% obese=102% overweight and obese) HORMONAL STUDIES in women only- percentages given in paper may not be correct (study design and testing does not mention why only 36 women were selected for biochemical testing however in discussion authors state: " exclusion of women with severe acne, hirsutism or menstrual disorders": no details and numbers provided) 36/109 (33%) tested for 17b estradiol, DHEAS, SHBG, testosterone, androstenedione, androgen index and progesterone. Author conclusion: no significant differences between HS women patients and 14 sex matched controls for 17b estradiol, DHEAS and SHBG levels. States there are statistically significant differences in testosterone, androstenedione and androgen index between the no flare versus flare group and the no flare versus controls. Median values for flare plus no flare not recorded in table and compared with controls (' androgen levels in most patients fell within the normal rangeTestosterone 78%, androstenedione 89%) Subnormal progesterone levels were recorded in 11/36 (30.5%) HS women tested. (NOT IN TABLE) Of these, 9/36 (25%) HS women were in the without premenstrual flare group and 2/36 (.05%) were in the premenstrual flare group. Statistical significance is attached to these subnormal progesterone figures using a different significance test to the androgen table of data. No figures given to compare to controls. Conclusion: "An increase in symptoms during the premenstrual phase was reported by 57% of women" The actual reported figure for premenstrual flare is 47/109 or 43%. 1989 Body Weight in HS Jemec.pdf Jemec GB. Body weight in Hidradenitis Suppurativa. In: Marks R, Plewig G (eds). Acne and Related Disorders. London: Martin Dunitz, 1989:375-376. Study undertaken to discover if HS is a complication of obesity. height and weight assessed in 76 patients referred for surgical treatment of HS and compared to ideal body weight charts. No significant difference was found. Male:Female=6:70. Axillary disease in five, genitofemoral disease in 37, combined axillary-genitofemoral disease or involvement of other areas 34 patients. Duration of disease between 1 and 43 years. 1988 Symptomatology of HS Women Jemec.pdf Jemec GB. The symptomatology of hidradenitis suppurativa in women. Br J Dermatol. 1988; 119(3):345-350. In order to establish the possible influence of androgens in patients with HS, information on other symptoms of androgenization was obtained from a group of 70 female patients with HS. Patients referred for surgical treatment of HS were questioned. The diagnosis of HS was based on the clinical presentation at the time of operation, and a history of recurrent and chronic inflammation, recurring at the same place in the groin, under the arms, between the buttocks, or on the breasts, the lesions suppurating, and unresponsive to normal treatment for boils, such as lancing. A control group was chosen from female hospital staff and from patients referred for cosmetic excision of naevi or warts. None of the women in the control group had any personal history of HS or any other infection involving the axillae, submammary region or groin. IN THE CONTROL GROUP 4 WOMEN WERE FOUND TO HAVE HS AND WERE TRANSFERRED TO THE STUDY GROUP. THIS WOULD SUGGEST A PREVALENCE OF APPROXIMATELY 4% IN THE GENERAL FEMALE POPULATION. 70 women with HS, mean age 31 years(range 17-59), mean age at onset of disease 23 years (range 6-58 years0. Localization of disease: 49/70 patients had disease in one region only Inguinoperineal 59, axilla 37,6,perianal 6 RESULTS: (?self-reported) Acne, hirsutism, irregular menstrual periods-no significant difference between HS and controls 18/70 (25.7%) positive family history of one or more cases of hidradenitis. 2/96 The menstrual cycle in HS patients significantly shorter than that of general female population between 23 and 45 years of age and the duration of the menstrul flow was found to be significantly longer in HS patients. 33/65 experienced exacerbation of disease during menstruation. TABLE 2 CHANGES IN THE SEVERITY OF HS DURING AND AFTER PREGNANCY NO OF PATIENTS 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 42 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database BETTER UNCHANGED DURING PREGNANCY 9 17 POST-PARTUM 2 15 *Three patients were pregnant at the time of interview (NO RECORD of OCP USE in patients and controls) WORSE 6 11 TOTAL* 32 28 1988 Endocrine basis for HS Harrison.pdf Harrison BJ, Read GF, Hughes LE. Endocrine basis for the clinical presentation of hidradenitis suppurativa. Br J Surg. 1988; 75(10):972-975. Examines specific, clinical and biochemical features of patients to assess the relationship between the clinical presentation of HS and the "putative" underlying endocrine (androgen) disorder. The clinical evidence obtained from first study guided the direction of the biochemical study. Case records for 152 patients with HS examined-referred to the Uni Dept of surgery between 1978 and 1986. STUDY 1 Age at onset-110 patients, 66% of men and 72 % of women experienced first symptoms before the age of 31 years. Gynae/obstetric features-87 women Sites involved-146 patient records followed up with 130 interviews. HS affecting axillary and inguinoperineal skin 83 %, HS affecting perianal skin 12%, HS affecting mammary skin 10%. Sex rate: axillary was the same in both sexes, inguinoperineal involvement 71% men, women 66%. Mammary HS, women 13%, men 2%. Perianl disease, men 37% versus 3% in females. Multiple site disease observed in 56% of cases, both sexes equally frequent. Acne- the prevalence of significant acne in men with HS was 42% and in women acne 27% of women. STUDY 2 Venous blood samples 36 female patients, 14 controls. No differences in estradiol and SHBG and DHEAS. Serum levels of testosterone, androstenedione and the abdrogen index were significantly greater in women from the no flare group than controls and women with a premenstrual flare. Serum progesterone values were significantly lower in women without pre-menstrual flare. Subnormal progesterone values in 9 women 78% OF PATIENTS HAD NORMAL RANGE VALUES OF TESTOSTERONE / 89% HAD NORMAL RANGE VALUES OF ANDROSTENEDIONE TABLE 1 AGE AT ONSET OF HS IN 35 MEN and 75 WOMEN AGE MEN WOMEN TOTAL% 0-10 1 3 3.6% 11-20 9 17 23.6% 21-30 13 34 42.7% 31-40 12 15 24.5% 41-50 5 4.5% 50+ 1 <1 TABLE 2 PREMENSTRUAL AND GYNAECOLOGICAL STATUS IN 83 WOMEN WITH HS 'Flare' Regular cycles 41 Irregular cycles 6 Total 47 'No flare' Regular cycles 6 Irregular cycles 19 Plus others Oral contraceptive users 8 Perimenopausal 2 Peripubertal 1 Total 36 TABLE 3 FREQUENCY DISTRIBUTION OF 'Overweight' and "obese' body mass index scores with HS and in population-based controls HIDRADENITIS CONTROLS Men n 31 patients 217 controls Overweight 77%* 44% 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 43 Annotations MSAB Database Obese 26%* 7% Women n 78 patients 247 controls Overweight 69* 33% Obese 33* 7% *P<0.001;x2 test (HS versus controls) TABLE 4 MEDIAN (MINIMUM TO MAXIMUM) values of testosterone, androstenedione and androgen index in women with HS NO FLARE FLARE CONTROLS Testosterone 1.9(0.6-4.1)* 1.5(0.8-3.2) 1.5(7-2.6) Androsteniodione 6.2(2.1-13.4)* 4.4(2.9-8.7) 3.7(2.9-9.7) Androgen Index 3.5(0.6-9.7)* 3(1.7-5.4) 2.4 (1.2--4.4) *P<0.05; Mann-Whitney U test; no flare versus flare, and no flare versus controls 1988 AN IR Cutaneous virilism Barth.pdf Barth JH, Ng LL, Wojnarowska F, Dawber RP. Acanthosis nigricans, insulin resistance and cutaneous virilism. Br J Dermatol. 1988; 118(5):613-619. 227 patients assessed for hirsuties. 74 were selected and agreed to prolonged anti-androgen therapy. Three had HS. Additional two patients with HS added to this study from...? Majority of 74 had AN of axilla and inner thighs. The more obese subjects had An in intertrigenous areas under the breasts and in the fatty fold. Metabolic profiles taken. Treatment with anti-androgens unsuccessful for AN in this group of patients. 1986 Trial cyproterone for HS Mortimer.pdf Mortimer P, Dawber RP, Gales MA, Moore RA. A double-blind controlled cross-over trial of cyproterone acetate in females with hidradenitis suppurativa. Br J Dermatol. 1986; 115(3):263-268. Two treatments trialed for 24 females, double blind cross-over study. Both Cyproterone acetate/ethinylestradiol (CPA) and ethinylestradiol/norgestrel (E50) are anti-androgenic and reduce circulating androgens. Assessment of patient condition and treatment effect was problematic. 6 patients dropped out of study (drug intolerance, 2 for each preparation) and two dropped out for treatment failure. 18 completing trial, five patients improved from baseline, four remained the same and two deteriorated. Seven patients cleared-having the disease from 5-20 years. 1986 Mediation of HS by androgens Mortimer.pdf Mortimer P, Dawber RP, Gales MA, Moore RA. Mediation of hidradenitis suppurativa by androgens. Br Med J (Clin Res Ed). 1986; 292(6515):245-248. We undertook to study the hormonal, and in particular androgenic influences in HS. From a series of 42 women we documented clinical data and examined hormonal biochemistry in an attempt to evaluate the endocrine basis of this disease.. PATIENTS AND METHODS: 42 women with recurrent "blind boils" in skin bearing apocrine glands were assessed for signs of androgen excess. CLINICAL DATA: 42 women aged 15-59 mean age 32. Disease activity six months to 27 years (mean 8.5 Years). Age at onset from 11 to 43 years (mean 23.3 years, median 19 years): 25/42 aged 11-20, six aged 21/30,seven aged 31-40 and three aged 41-50. Axillary disease 9/42, Genitocrural sites were affected 15/42, both sites affected 18/42. Other sites affected included 3/42 submammary cases, 1/42 perineum and buttocks, 1/42 labia, 1/42 ear lobes 13/42 noted irregular menses(more than five days adrift from expected date). (control data on prevalence of irregular menses show wide normal variation depending on age, making conclusions difficult to draw). 22/36 noted exacerbation, enlargement, and increased tenderness of abscesses with or without increased purulent discharge immediately before the start of menses. 6/42 had hysterectomies but not oophorectomies. No patients showed evidence of systemic virilization. 19/42 (45%) had or had had acne vulgaris compared with under 10% in a control population. 7/42 (16%) were hirsute (>10 Ferriman and Gallwey grading system) compared with under 2% in a control population Comedones-open comedones found in 37/42, usually in diseased areas, but also in distant sites bearing apocrine glands that were unaffected by HS. 18/42 (42.8%) evidence of retroauricular comedones Pilonidal sinus 8/42 (19%) had evidence of PS-all had prior surgical treatment for PS, 2/42 suffered recurrence of PS (Author compares incidence of PS to Mayo clinic stat of 1:3700 {.02%) incidence of PS (1962) The fairly strong strong association in our patients suggests that PS and HS are related diseases, sharing the same basic pathogenesis, that is apopilosebaceous blockage. BIOCHEMICAL FINDINGS: in 41 samples from women with HS, 25 "normal" women as controls matched 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 44 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database for age, 37 women with idiopathic hirsutism as additional control matched for age No significant difference between three groups for SHBG, prolactin, thyroxine, FSH, LH, 17b oestradiol. (Distribution and ranges of SHBG were different) Following all within normal ranges in 42/42 HS women- hemoglobin, glucose, IgG concentrations and white cell count Plasma testosterone concentration was significantly higher in HS patients and hirsutism patients than controls. Distribution and ranges of concentrations of T in patients with HS and hirsutism were different from controls. The free androgen index (ratio of T to SHBG concentration) was significantly higher in HS and Hirsutism patients _____________________________________________________________________________ TABLE OF MEDIANS measured in (nmol/l) GROUP STUDIED SHBG Testosterone Free Androgen Index DHEAS Controls n=21 51.5 1.1 .019 5.1 HS n=41 54 1.7 .029 5.5 Hirsutism n=37 45 2.6 .06 7.1 ___________________________________________________________________________ Discussion: None of our patients had evidence of systemic virilization. Many cases of the chronic debiltating form are the result of failure to diagnose and treat eraly disease. Awareness of its existence and, more importantly, recognition of initial clinical features are the main reasons for this error. We suggest the following clinical criteria for diagnosis 1.recurrent deep seated inflammatory nodules in skin bearing apocrine glands persisting for a minimum of three months, not always discharging or fluctuating, and for a tendency for cord like coalescence and 2.comedones in skin bearing apocrine glands and the ear lobes. Minor criteria include an association with acne vulgaris and, in women, exacerbation with menses. 1986 HS cyproterone estrogen Rx 4 women Sawers.pdf Sawers RS, Randall VA, Ebling FJ. Control of hidradenitis suppurativa in women using combined antiandrogen (cyproterone acetate) and oestrogen therapy. Br J Dermatol. 1986; 115(3):269-274. Aim: Since acne, an androgen-dependant condition rsponds to combined cyproterone acetate and oestrogen therapy we have investigated the effect of such treatment on four women with long-standing HS. The authors reported in 1980 of one women with HS who had responded to this therapy Patients: Age at start of RX Location of HS Duration of HS Patient 1 33 Vulval, pubic 5 years Patient 2 39 Groin axilla 'Since childhood' Patient 3 29 Vulva, ear canal 19 years Patient 4 24 Vulva 5 years All four women had at least one child and none was hirsute. Previous RX Patient 1 Antibiotics, radiotherapy Patient 2 Antibiotics Patient 3 Ovran (OCP), surgery Patient 4 Ovranette (OCP) ceased prior to study Investigations: Sebum production measured. Hormone levels taken (endocrine assays reported 1982 (Sawers, Randall, Iqbal) Results "The condition was controlled successfully in all patients with 100 mg/day cyproterone acetate using the reversed sequential regimen; lowering the antiandrogen to 50 mg/day caused deterioration." 2 out of four suffered recurrence. Sebum production in patients 1 and 3 was initially high but was rapidly reduced after one to two cycles of treatment and remained low. Patients 2 and 4 had normal sebum production rates both before, and during therapy. Before treatment, plasma testosterone levels were within the normal range, but plasma androstenedione values were raised and sex hormone binding globulin levels were low. On treatment, the androstenedione concentration fell and sex hormone-binding globulin values were raised. However, since these levels were unaltered by reducing the antiandrogen dosage, the main action of the therapy is probably that of the antiandrogen within the target cells. Side effects All four patients reported feeling depressed during treatment. Patient 2 withdrew from therapy after 13 months due to depression, patient 3 became ammenorrhoic when treatment was discontinued. No consistent effects on weight were noted. 1986 HS an androgen dependant disorder Editorial Ebling.pdf 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 45 Annotations MSAB Database Ebling FJ. Hidradenitis suppurativa: An androgen-dependent disorder. Br J Dermatol. 1986; 115(3):259-262. Editorial summarizing recent developments. 1985 HS endocrine abnormality Harrison.pdf Harrison BJ, Kumar S, Read GF, Edwards CA, Scanlon MF, Hughes LE. Hidradenitis suppurativa: Evidence for an endocrine abnormality. Br J Surg. 1985; 72(12):1002-1004. A study was undertaken to investigate the hormonal background of HS. Thirteen women with HS who all experienced a premenstrual exacerbation of their disease and 9 age matched female volunteers were studied on an outpatient basis. 1.17B oestradiol and progesterone basal levels were within the normal luteal phase range in patients and controls: no significant differences. 2. Testosterone and DHEAS no significant differences. Two patients plasma testosterone 3.16 nmol/l and 2.66nmol/l (normal range 0.3-2.5nmol/l 3. Mean basal levels TSH no significant difference between patients and controls. Basal levels of free T3 and free T4 no significant difference between patients and controls. Following TRH stimulation/challenge TSH levels significantly greater in HS group at 20min and 60min. 4. Mean basal levels of prolactin no significant difference between patients and controls. Three patients in HS group had minimally elevated basal prolactin levels at 476, 565 and 453 mU/l (normal range ,420 mU/l). Stimulated PRL levels following TRH stimulation/challenge significantly higher in the HS group than the controls at 10min and 20min. 5. Mean basal levels of LH and FSH were within normal ranges in both groups. 1 patient had an LH/FSH ratio .2 Following stimulation/challenge with LH-RH, mean LH and FSH levels were not significantly different between the HS and control groups. Discussion-"failed to demonstrate uniform elevation of serum testosterone in HS patients. Prolactin has a marked circadian ryhtm and pulsatility of secretion and the TRH stimulation test therefore provides a more accurate assessment of prolactin storage pools than does a single random basal level. An exaggerated prolactin response to TRH indicates increased releasable stores of TSH...these responses to TRH may well reflect increased oestrogenic activity rather than a primary disorder of the hypothalamopituitary axis or of thyroid function. There was no clinical or biochemical evidence of hypothyroidism in these patients." 1971 HS and pregnancy Woods.pdf Woods G, Vogel EH, Croak J. Hidradenitis suppurativa and pregnancy. case report and discussion. Ohio State Med J. 1972; 68(9):864-866. A case of HS during pregnancy is presented, which worsened following delivery. Because the perineum was markedly involved with small abcess and dense fibrous tissue from scarring, caesarean section rather than vaginal delivery was felt to be indicated. Pregnant patients with severe HS of the vulva should be delivered abdominally. 34 year old women presents with HS of the vulva. Four years prior to vulvar involvement she had HS of both axillae which was treated by surgical ecision and SSG with good results. Past medical hx or hemorroidectomy and fistulectomy some ten years ago. The vulvar HS worsened, the patient became pregnant. At 36 weeks she was evaluated for worsening HS which would complicate a vaginal delivery. Patient resistant to treatment for anemia prenatally. Cultures of vagina showed no growth. Cultures of vulva pustules grew Staph Epidermis and alpha-streptococci sensitive to cloxacillin. Patients vulval HS resistant to dicloxacillin (oral), iodochlorhydroxyquin (antibacterial/antifungal topical),hydrocortisone, burrows solution, sitz baths and prednisone. Three months after delivery the woman's vulval HS worsened and this was surgically treated. 9 Surgery 2005 Surgery for HS 10 year review Kagan.pdf Kagan R, Yakuboff K, Warner P, Warden G. Surgical treatment of hidradenitis suppurativa: A 10-year experience. Surgery. 2005 Oct; 138(4):734-41. “Dr. Kagan reviewed the university hospital's experience with 92 surgical procedures in 57 patients with the condition treated from 1994 to 2003. Most of the patients were female (72%) and African American (70%) with a mean age of 34 years. They had hidradenitis suppurativa for nearly 7 years on average... Axillary disease occurred in significantly more women than men (88% vs. 13%, respectively) while inguinoperineal disease was significantly more common in men than in women (87% vs. 12%, respectively).” 2005 Experience with different treatment modules 106 HS patients Mandal.pdf 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 46 Annotations MSAB Database Mandal A, Watson J. Experience with different treatment modules in hidradenitis suppuritiva: A study of 106 cases. Surgeon. 2005; 3(1):23-26. The study analysed 143 sites in 106 patients with hidradenitis suppuritiva treated surgically in the regional plastic surgery unit of southeast Scotland over a period of 11 years (January 1990 to December 2000). Study describes a 70% recurrence rate following primary closure of 100 HS surgical sites, although no details are given to the specific location of the recurrence in relation to the excision site. The authors advise that primary closure of these surgical sites leads to unacceptable risk for recurrence and re-operation. This paper is essential reading for surgeons who want to choose the best method of surgical excision and closure. This paper is essential reading for HS patients considering surgical options. Females 88.78%, males 11.22%. "The median age was 36 years (range 11-70 years). The patients were..heavy smokers (98.1%). The disease primarily involved the axillary region bilaterally followed by unilateral axillary insolvent and the inguinoscrotal region Median surface area was 15cm2 The ‘mild’ group contained 64 patients and the ‘severe’ group 42 patients. Median interval between onset of the disease and active surgical intervention was six years (range 1-30 years).” Co-morbid medical disease: Diabetes mellitus 13 patients 12.2% Asthma 5 patients 4.2% Hypothyroidism 2 patients 1.8% Crohn’s Disease 2 patients 1.8% Arthritis 2 patients 1.8% Depression 2 patients 1.8% 2005 ABSTRACT Therapy for HS 2000 patients AAD Wienert.pdf Wienert V. Therapy for advanced acne inversa: 30 years of experience in almost 2000 patients, ABSTRACT . J Am Acad Dermatol. 2005 March; 52(3 Supplement 1):P155. Over a period of 30 years the author describes ‘almost’ 2000 patients treated for Hidradenitis Suppurativa. No total gender statistics are provided. “The average age of disease manifestation among male patients was at 28 years, among female patients at 23 years. The oldest patient who manifested the disease was 53 years of age; the youngest one, 14 years. The distribution of the localization of skin symptoms included the perianal, inguinal, and axillary regions but also gluteal, perigenital, submammary, and umbilical zones as well as the thighs. All patients had previously received conservative or surgical therapy without success.” 2005 Abstract HS Sx management Krstic.pdf Krstic V, Kozarski J, Panajotovic L, Djordjevic B, Gacevic M, Stanojevic B. Presentation abstract OP015 Hydradenitis suppurativa - Surgical management, 10th ESPRAS congress <http://www.espras.org/upload/AbsOrals.pdf>. Accessed 2/20/06. The European Society of Plastic Reconstructive and Aesthetic Surgery, Vienna, Austria, 2005 Sep. Hidradenitis suppurativa is a chronic recurrent inflammatory process involving apocrine sweat glands and adjacent connective tissue. It is manifested by acute abscesses, persistent pain and sinus tract and fistula formation in a chronic phase. Matherials and methods: Over the past 10 years 36 patients ( 27 male and 9 female ), with this process have been treated in our hospital. The axillary (20%) and inguinoperineal (45%) regions are the most commonly affected areas. Most of them were initially treated conservatively. Results: Surgical treatment consisted of wide ablative en-bloc excision of affected skin and subcutaneous tissues, and primary closure of the defect with direct sutures in 16 (44%) cases, skin graft in 27 (44%) cases or local flap, in 9 (25%) cases. Local flaps have been used exclusively in the axillary region 5 (13,8%). About 50% cases have been reoperated because of the disease propagation or its occurence in a new localisation. Conclusion : Radical excision of the affected regions is the basis of treatment of this disease. The method of defect closure is determined depending on the localisation and extensity. Our opinion is that the results are functionally and aesthetic better and the frequency of disease recurrence reduced when local flaps are used for coverage. 2004 Surgery Axillary HS Altmann.pdf Altmann S, Fansa H, Schneider W. Axillary hidradenitis suppurativa: A further option for surgical treatment. J Cutan Med Surg. 2004; 8(1):6-10. 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 47 Annotations MSAB Database Between 1996 and 2000, 20 patients with axillary hidradenitis suppurativa were treated and followed up retrospectively between 3 months and 5 years. There were 14 females and 6 males with an average age of 36 years (range = 20–50 years). Fifteen patients were smokers. Duration of the disease prior to surgery was between 2 and 8 years. In most cases, incision of abscesses was performed prior to admission. At the time of admission seven patients had a putrid productive infection with staphylococci. The mean postoperative hospital stay was 8 days. Approximately 25% complication rate post-op. 2003 POSTER Wound care post surgical excision HSWounds Uk Enoch.pdf Enoch S, Bhattacharya L, Robert BR, et al. Meticulous wound care after surgical excision expedites healing and prevents recurrence of Hidradenitis Suppurativa Page 11 <http://woundsuk.com/posterabstracts2003.PDF>. Accessed 4/23/2005. Wounds-UK, United Kingdom, 2003. The aims of our study were two fold: (i) to identify the optimal post-operative (post excision) management and to ascertain the role of regular wound care and (ii) to evaluate if healing by secondary intention is effective in the management of HS. Methods We undertook a six-year retrospective review (1995 – 2000) of the case notes of all patients who presented to our unit after surgical excision of HS. Results From a total of 66 patients identified initially, a final analysis was performed on 62 patients (F: 46, M: 16; Mean age: 38.4) with 85 wounds. Four patients were lost to follow up. 2003 Gluteal and perianal HS Surgical rx Bocchini.pdf Bocchini SF, Habr-Gama A, Kiss DR, Imperiale AR, Araujo SE. Gluteal and perianal hidradenitis suppurativa: Surgical treatment by wide excision. Dis Colon Rectum. 2003 Jul; 46(7):944-9. Colorectal Unit of the Gastroenterology Department at the University of Sao Paulo Medical School between January 1980 and May 2000 were reviewed...56 patients underwent treatment of hidradenitis suppurativa in gluteal, perineal, and inguinal areas through wide excision; 52 (93 percent) were male and 36 (64 percent) were white.” 7% female. “Serum levels of testosterone, hydroxyprogesterone, and dehydroepiandrosterone were normal in the 21 patients undergoing such studies.” “Diverting colostomy was used in 23 (41 percent) patients because of extensive and complex lesions involving perianal margins.” ASSOCIATED CONDITION NUMBER % Perinal fistula 8 14.3% Acne 6 10.7 Diabetes mellitus 2 3.6 Acne conglobata 1 1.8 Squamous-cell carcinoma 1 1.8 Gout 1 1.8 Crohn’s disease 1 1.8 2002 CO2 Laser Surgery HS retrospective Lapins.pdf Lapins J, Sartorius K, Emtestam L. Scanner-assisted carbon dioxide laser surgery: A retrospective follow-up study of patients with hidradenitis suppurativa. J Am Acad Dermatol. 2002; 47(2):280-285. MINIMALLY INVASIVE TISSUE SPARING TECHNIQUE OF TREATING/REMOVING SYMPTOMATIC LESIONS 35 patients (31 females) underwent surgery for HS in our department between 1993 and 2000 were analyzed retrospectively. The mean duration of disease was 13.4 years (range, 1-35 years). Previous therapy included injections of and multiple courses of antibiotics (18 patients); local incisions (n 20), isotretinoin (n 3); isotretinoin and cyproterone acetate, followed by finasteride (n 1); excision surgery (n 2); and excision surgery with transplantation (n 1). Five patients had undergone previous successful in loco carbon dioxide laser surgery before 1993 in our department. Table I. Baseline characteristics of the 34 patients treated for hidradenitis suppurativa with scanner assisted carbon dioxide laser surgery Variable Value* Age (y) 33.9 11.0 Sex (M/F) 3/31 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 48 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database Age at onset (y) 22.3 6.6 Disease duration (y) 13.4 8.6 Acne: past or present (yes/no) 16/18 Hereditary/nonhereditary variant† 11/23 Smoker/nonsmoker‡ 25/9 Children (yes/no) 16/18 Follow-up (mo) 34.5 25.7 *Plus-minus values are means standard deviation. †Family history of hidradenitis suppurativa in one or more first-degree relatives reported by the patient. ‡Smoking reported by the patients at the time of surgery. All but one of them were pleased with the appearance of their scars after complete wound healing. Patients who had undergone other surgical methods for this condition preferred our laser method, and all patients but one would have accepted the same type of laser surgery again, if necessary. 2000 Surgical treatment Acne Inversa University of Heidleberg Kurzen.pdf Kurzen H, Schönfelder-Funcke S, Hartschuh W. Surgical treatment of acne inversa at the university of heidelberg. Coloproctology. 2000; 22:76-80. "We evaluated 66 patients with acne inversa, treated in our clinic between 1987 and 1999....both sexes equally frequent" 2000 Longterm results surgery HS Rompel.pdf Rompel R, Petres J. Long-term results of wide surgical excision in 106 patients with hidradenitis suppurativa. Dermatol Surg. 2000; 26(7):638-643. "106 patients suffering from HS treated in the Department of Dermatology, Klinikum of Kassel, Germany during the period 1980-1989, 57.5% women, 42.5% men" 2000 Chronic axillary HS wide excision flap coverage Soldin.pdf Soldin MG, Tulley P, Kaplan H, Hudson DA, Grobbelaar AO. Chronic axillary hidradenitis--the efficacy of wide excision and flap coverage. Br J Plast Surg. 2000; 53(5):434-436. 2000 Combined SA/UK STUDY 2000 Capetown, South Africa “Forty patients (64 axillae) were treated at Groote Schuur Hospital over a 14-year period (1982–1995).” (42 females, 17 males combined study with groote Mount Vernon Hospital below) 71% female, 29% male 2000 Middlesex, UK. “Nineteen patients (30 axillae) were treated at Mount Vernon Hospital (1992–1998).” (42 females, 17 males combined study with groote Schuur Hospital above) 1998 Extent of surgery recurrence rate Ritz.pdf Ritz J, Runkel N, Haier J, Buhr H. Extent of surgery and recurrence rate of hidradenitis suppurativa. Int J Colorectal Dis. 1998; 13(4):164-168. “Medical records, including outpatient files, of all patients who underwent surgery in our Department between 1976 and 1997 were analyzed retrospectively... Surgery for HS was performed in 31 patients (8 women and 23 men).” 26% female, 74% male.” 1998 Early surgical treatment perianal HS Endo.pdf Endo Y, Tamura A, Ishikawa O, Miyachi Y. Perianal hidradenitis suppurativa: Early surgical treatment gives good results in chronic or recurrent cases. Br J Dermatol. 1998; 139(5):906-910. Presentations of perianal HS to University department of Dermatology April 1986 to March 1997 (10 males 83%, 2 women 17%) Includes 1 patient with axillary HS , 1 patient with HS ‘nape of neck’ 1996 Co2laser Rx HS Finley.pdf Finley EM, Ratz JL. Treatment of hidradenitis suppurativa with carbon dioxide laser excision and second-intention healing. J Am Acad Dermatol. 1996 Mar;34(3):465-9. 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 49 Annotations MSAB Database Background: Hidradenitis suppurativa is a disabling disease. Although medical management can at times control the disease, the treatment of choice is surgical excision. Surgical defects have been managed by second-intention healing, simple surgical closure, or complex surgical repairs. Objective: Our purpose was to determine the benefit of carbon dioxide laser excision with healing by second intention to treat lesions of hidradenitis suppurativa. Methods: We treated seven patients who had axillary and inguinoperineal hidradenitis suppurativa with carbon dioxide laser excision followed by second-intention healing. A total of 12 procedures were performed (five bilateral and two unilateral). Follow-up periods were 10 to 27 months. All areas were excised using CO2 laser surgery with the patients under local anaesthesia with conscious sedation. Three patients procedures were performed as day surgery in a hospital operating room, four patients procedures performed in outpatient surgery suites. All diseased areas were excised with a margin of 3-4mm beyond clinically involved tissue as determined by visual inspection and palpation. The dissection was carried out deep to abscesses and sinus tracts. Blood loss was negligble and absolute hemostasis was obtained with the laser in a defocused mode and with spot electrocoagulation when necessary. Treated areas healed by second intention. Wound care once or twice daily cleansing with hydrogen peroxide followed by application of bacitracinpolymixin B antibiotic ointment and a Telfa dressing. Ketorolac was used for intraoperative and postoperative analgesia 60 mg intramuscularly and 10mg by mouth respectively. Four patients received 1gm of erythromycin by mouth per day, whereas three received no antibiotic coverage. Of the seven patients, two had bilateral procedures on the same day, three had bilateral treatment with each side treated at approximately 4-6 week intervals, and two had unilateral treatment. After excision patients performed full range-of-motion exercises to prevent contractures. Patients: Age range 20-46 years. Three had axillary disease and four inguinal. Duration of disease ranged from 1 to 10 years. All patients had received multiple courses of antibiotics and topical antiseptics; two had received intralesional corticosteroids, and three had received intralesional corticosteroids, and three had received courses of isotretinoin. Three patients had had previous excisions of symptomatic nodules. One patient had well-controlled thyroid disease and glaucoma, and the remaining six had no other medical problems aside from HS. One patient was taking oral contraceptive. Three patients had received oral isotretinoin for four to five months before undergoing excision. Complications: One patient had temporary paresthesias, and minimal axillary stricture 3 months after operation after prematurely discontinuing the recommended exercises. The stricture resolved when the exercises were resumed. One patient required manual debridement of an inguinal wound because daily local wound care had not been adequate. In other patient candidiasis surrounding an inguinal wound responded to 400mg of oral ketoconazole for two weeks. Results: -------------------------------------------------------------------------------------------TABLE 1 CO2 laser excision sites and time to wound healing No. Site Wound surface area (cm2) Time to complete healing(wk) 1 R axilla 45 11 L axilla 33 8 2 R inguinal 60 8 L Inguinal 15 7 R Labia 4 7 3 L groin 10 4 R groin 21 5 4 R axilla 58.5 7 L axilla 15 4 5 R axilla 54 7 6 L groin 22.5 6 7 L groin 10 6 R groin 10 6 ______________________________________________________________ After a healing time of 4 to 11 weeks, wounds contracted and scars were flat and linear. Patients were observed for 1027 months. Only one recurrence of hidradenitis suppurativa has been observed; this occurred along the margin of the surgical scar in a previously treated area. No correlation was seen between duration of disease and recurrence.At followup five patients had continued disease activity in untreated areas. All subjects were satisfied with the results of the procedure, and complications were minimal. 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 50 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database Conclusion: Carbon dioxide laser excision with second-intention healing is safe and effective for hidradenitis suppurativa. Surgical intervention in patients with HS should not be delayed because squamous cell carcinoma has been reported in 16 patients. Seven of these died of widespread metastatic disease. 1991 CO2 Laser Vulval HS Sherman.pdf Sherman, AI. Reid, R. CO2 laser for suppurative hidradenitis of the vulva. J Reprod Med. 1991 Feb;36(2):113-7. Prolonged antibiotic therapy and multiple incisions and drainage have been the usual form of therapy, but those surgical procedures leave patients with ugly scars; retracted, hypertrophic skin edges; and pitted, indurated and disfiguring abscesses. The only successful treatment to date has been wide excision of the entire apocrine gland-bearing tissues, such as radical vulvectomy, which often necessitates grafts to cover the surgically exposed areas. That has left patients with unsatisfactorily functioning vaginas and a mutilated appearance. With the intent of avoiding mutilating results, we recently instituted the use of the CO2 laser to treat these patients with the goal of eradicating the suppurative sinus tracts and the infected apocrine glands without removing large volumes of adjacent, unaffected tissue. Over the past eight years we treated 11 patients who had extensive, chronic hidradenitis of the anogenital area with the CO2 laser. The use of laser therapy for these patients has produced effective and less-mutilating results. Such treatment has been a successful option for treating this debilitating disease. The tendency towards a genetic predisposition and family history was evident; there was one mother-daughter combination, and two other patients were sisters -------------------------------------------------------TABLE 1 Distribution of Disease (edited) Patients age Location of HS 19 Vulva, Perianal 23 Vulva, Perianal, Symphyseal area 27 Vulva, Perianal, Symphyseal area 31 Vulva, Perianal, Buttocks, Symphyseal area 29 Vulva, Perianal, Buttocks, Symphyseal area 43 Vulva, Perianal, Buttocks, Symphyseal area 51 Vulva, Perianal, Symphyseal area 34 Vulva, Perianal 37 Vulva, Perianal, Symphyseal area 41 Vulva, Perianal, Buttocks, Symphyseal area 29 Vulva, Perianal TABLE 2 Number of Laser Treatments and duration of recovery No. of treatments Length of hospital stay Time from treatment to healing 1 1 day 2 weeks 1 1 3 1 1 3 2 4 4 2 9 6 2 8 8 1 1 5 1 1 3 1 2 3 1 5 3 1 3 2 1988 Effect localized surgical excisions HS Jemec.pdf Jemec GB. Effect of localized surgical excisions in hidradenitis suppurativa. J Am Acad Dermatol. 1988; 18(5 Pt 1):1103-1107. This study was undertaken in order in order to follow the effects of wide surgical excision of HS followed by primary closure Materials and methods; 84 patients treated for HS in the period 1975-1986 were included in the study-all patients treated with wide surgical excisions with primary closure-all treated in an outpatient setting. Information obtained from 72/84 (85.7%) patients treated (two patients had died by time of review, four could not be traced and six did not want to participate in the study) Results: 66/72 female(91.7%), 6/72 male (8.3%). Average age at onset of disease 23.8 +/_ 10.6 years with a range of 6-52 years. Pre-op duration of disease mean 4.5 +/_ 3 years with a range of 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 51 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database 1 to 11 years Localization of disease in 72 patients- inguinoperineal 60, axillary 26, mammary 6, perianal 7 Complete cure in 14.7%; Cure in treated region but subsequent recurrence in another region-7.4%; Disease weakened somewhat after surgery-2.7% Discussion: There seems to be a consensus in the literature that simple incisions are of no value in the treatment of HS. The extent of surgery needed is, however, disputed, and while some authors find that simple wide excisions with primary closure are sufficient, others advocate more extensive excisions involving the entire area potentially harboring apocrine glands. From this study it is clear that limited surgery alone does not offer a universal cure of hidradenitis.Cure does not seem to be associated with the localization of the disease or preoperative duration, but the group of patients who were cured were significantly older at the onset of the disease (p less than 0.001) than the group with recurrent disease. Patient satisfaction with excisional surgery was high, with 68% reporting surgery to be superior to other types of treatment experienced and only 6% considering it worse. It is hypothesized that the rapid relief, even if offered temporarily, in an otherwise chronic disease is the cause of this high patient satisfaction. 1987 Recurrence of HS post surgery Harrison.pdf Harrison BJ, Mudge M, Hughes LE. Recurrence after surgical treatment of hidradenitis suppurativa. Br Med J (Clin Res Ed). 1987; 294(6570):487-489. Typical features of long-standing disease are chronic sinus formation, subcutaneous fibrosis, and a progressive disorganisation of normal skin architecture. purulent discharge from the deep, painful, recurrent lesions causes considerable social morbidity and distress. Many reports that describe the surgical treatment of hidradenitis lack detailed follow-up information, but when it is included the follow up is of short duration. We carried out a detailed review of patients who had undergone wide excision to assess the morbidity that results from surgery and the incidence of recurrent disease. Between Jan 1978 and June 1985, 95 patients with severe HS were treated by wide excisional surgery in the University Department of Surgery, Cardiff, UK. This surgery was only offered to those with advanced disease. All patients had undergone prolonged conservative treatment prior to surgery. RESULTS: Patients invited to attend for review and clinical questionnaire. 82 patients reviewed (62 women, 20 men) 118 operations Mean age 32.5 years (range 14-56), duration of disease mean 8.8 years (1-44 years range0. Mean duration for follow-up after surgery was 47 months (range 6-89 months. Healing times for the wounds that were allowed to heal by granulation were- -axillae median 11 weeks (range 7-30) -inguinperineal median 16 weeks (range 8-30) -perianal median 17.5 weeks (range-12-30) Recurrent disease-recurred locally at 21 sites in 19/82 (23%) patients developing from thee to 72 months (median 24 months). Sites of recurrence-15 after inguinoperineal surgery, 3 after axillary excision, three after mammary excision No local recurrence after operation for perianal disease. 6/19 patients had required further surgery by the time of review to control symptoms related to recurrence Two distinct patterns of recurrent disease: 1.Confined to the immediate vicinity of the scar (10 patients) usually solitary and typically occuring in the groin creases or the posteroinferior margins after inguinoperineal surgery; typified by deep sinuses and appreciable morbidity required further excision 2. Multiple lesions present (9 patients), ocurred either adjacent to previous excision sites or a few centimetres away; characterised by widespread, often superficial lesions, were more trivial and resulted in little morbidity At review 20/82 (24.3%) patients had developed the disease at an anatomical site that had been unaffected at the time of the initial surgery. Morbidity:- complications arose BEFORE discharge from hospital in 14 patients (12% of operative procedures). Failure of skin graft in 9 patients (45% of applied grafts). 1 case ischaemic necrosis,1 case leg vein thrombosis, 1 patient axillary vein thrombosis, postoperative anemia in 4 patients, transient oedema in two patients. Four cases late wound mordidity required intervention-two with hypertrophic scars, one with wound tenderness-all treated by local excisions Discussion-The terms "wide" and "radical" excision are poorly defined in published papers, and thus it is difficult to compare and evaluate the methods and results of different published papers, and thus it is difficult to compare and evaluate the methods and results of different published series. Emphasis is often placed on the technique used to cover an excision defect rather than the extent of excision or the success or failure of the treatment. Follow up has been short or of unspecified length, and often absent. Recurrence rates that are quoted without a reference to the site at which 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 52 Email: [email protected] - http://www.hs-foundation.org Annotations MSAB Database surgery was performed may mislead, and considering re-operation as a criterion of treatment failure will underestimate the number of patients with a less than satisfactory outcome. The need for prolonged follow-up, even after radical surgery, is apparent from or results. At review half of the patients with recurrence had developed these lesions more than two years after surgery. Just under a quarter of patients had developed hidradenitis at a previously unaffected site. 1982 Advanced HS Surgical case studies Broadwater.pdf Broadwater JR, Bryant RL, Petrino RA, Mabry CD, Westbrook KC, Casali RE. Advanced hidradenitis suppurativa. review of surgical treatment in 23 patients. Am J Surg. 1982; 144(6):668-670. “23 patients treated by the Surgical Services at the University Hospital and Little Rock Veterans Administration Hospital between 1967 and 1981” 14 male (61%), 9 female (39%) 10 Other treatments 2005 ALA-PDT for HS-no clinical improvement Strauss.pdf Strauss RM, Pollock B, Stables GI, Goulden V, Cunliffe.W.J. Photodynamic therapy using aminolaevulinic acid does not lead to clinical improvement in hidradenitis suppurativa. British Journal of Dermatology. 2005 April; 152(4):803. Four patients were enrolled, three with axillary disease and one with groin disease. Two patients reached an endpoint: one patient completed three treatments (patient 1), and the other noticed a significant improvement after two treatments, and no more treatments were therefore given (patient 2). At 8-week follow-up, however, the regional HS score had improved only marginally in one patient but had worsened in the other patient compared with baseline values. Of the two patients who did not complete the study, one received only one treatment and then decided against further treatments because of severe burning and stinging (patient 3). The fourth patient had two treatments and then decided against continuing therapy as the condition appeared to worsen (patient 4). Again, regional HS scores at follow-up in both patients did not show any improvement, but marked deterioration in one patient (patient 4). 2004 Transdermal CO2 application HS Wollina.pdf Wollina U, Heinig B, Uhlemann C. Transdermal CO2 application in chronic wounds. Int J Low Extrem Wounds. 2004 Jun; 3(2):103-6. Technique to improve perfusion of wound bed, decrease healing time. 17 patients with acute wounds, that is, wide excisions in the axillary and/or inguinal region in acne inversa, were included (5 females, 12 males, age range 29 to 42 years). 2004 Abstract Hyperbaric Oxygenation HS Gokulakrishnan.pdf Gokulakrishnan P, DeSylva P, Nangpal S, Sridhar J, Mohanty C. Hyperbaric Oxygen Therapy in Dermatology presented 17-19 November 45th Annual conference Indian Society of Aerospace Medicine <http://www.isamindia.org/conference44/papers.shtml>. Accessed 12/12/05. ISAM, Bangalore, India, 2004. Two cases HS responded well to HBO, no further details. 2004 (Abstract) Prognostic factors radiotherapy for HS Frohlich.pdf Frohlich D, Baaske D, Glatzel M. Prognostic factors of success of radiotherapy for hidradenitis suppurativa (Poster) <http://www.estroweb.org/ESTRO/upload/EVentsdocs/Non-MalignantDisabstractbook04.pdf>. Accessed 7/22/2005. 2nd ESTRO Meeting on Radiotherapy for Non-Malignant Diseases, Nice,France., 2004 Apr. We analysed the data of 293 patients suffering from hidradenitis axillaris (of these 142 were female and 151 were male). They had received radiotherapy in two different hospitals from 1979 to 2000. hidradenitis was clearly improved in 118 patients (40%). Abscesses were formed in 58 cases (20%). 1980 Silastic Foam dressing Rx of HS Morgan.pdf Morgan WP, Harding KG, Richardson G, Hughes LE. The use of silastic foam dressing in the treatment of advanced hidradenitis suppurativa. Br J Surg. 1980; 67(4):277-280. Study: 12 female 5 males with HS silastic foam used as adjuvant wound dressing treatment following surgery to increase re-epitheliazation, reduce healing times for healing by secondary intent. 1965 Xray epilation therapy HS Zeligman.pdf 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 53 Annotations MSAB Database Zeligman I. Temporary x-ray epilation therapy of chronic axillary hidradenitis suppurativa. Arch Dermatol. 1965; 92(6):690-694. A single dose of conventional superficial xrays resulting in temporary epilation has been found to be an effective treatment for chronic HS of the axillae in five cases. _____________________________________________________________ Patient characteristics AREA TREATED Duration of HS CASE 1 Male 43yo R axilla 6 months CASE 2 Female 28yo Bilateral axilla 3 years CASE 3 Female 38yo Bilateral axilla 7 months CASE 4 Female 28yo Right axilla 'Several months' CASE 5 Female 51yo Bilateral axilla 3 yrs 4 months ____________________________________________________________ Previous unsuccessful treatments Follow up times and treatment results: CASE 1 Incisions antibiotics 1yr and 6 yrs no recurrence CASE 2 compresses, ointments incision, avoid deodarants Erthromycin 3 months and four years no recurrence CASE 3 penicillin,staph toxoid, Approx 16 months no recurrence tolbutamide,sedatives avoid deodorants CASE 4 tetracycline Four yrs no recurrence CASE 5 tetracycline Four weeks incomplete success, recurrence at seven weeks approx 15 weeks no recurrence Nine months no recurrence (in all cases hyperpigmentation in previously affected areas still extent at follow up, 2/5 still had evidence of scar formation at follow-up.) 1950 Roentgen ray therapy HS Schenck.pdf Schenck SG. Hidradenitis suppurativa axillaris; an analysis of 54 cases treated with roentgen rays. Radiology. 1950; 54(1):74-7, illust. Diagnostic criteria very wide and patients included who had had "HS" for two weeks. HS occurs more frequently than is generally recognized and is often diagnosed as abscess, furunculosis, or carbuncle. This report is based on a series of 58 cases seen in the past ten years of HS axillaris. The author cites his own 1935 paper regarding roentgentherapy for cervical adenitis and proposes a mechanism of action for this treatment in HS: " the action of the rays on inflammatory processes is upon the round-cell infiltrate, consisting mainly of polymorphonuclear leukocytes and lymphocytes which are rapidly destroyed. This cellular destruction liberates the enzymes and antienzymes within the cellular walls which bathe the inflammatory process, thus neutralizing its toxins." 54/58 were followed for at least three months after the last treatment, this was the study population. Not included in this report are 4 cases involving the genitals and inguinal regions, 2 in the perianal area and, and 1 in the areola of the breast, all of which responded favorably to x-rays. 25/58 involved right axilla, 20 left axilla and 9 unilateral axillary disease. 54 cases in study Male to females was 25:29 age ranged from 15 to to 38, average being 24 years Duration of the disease when these patients first presented themselves for treatment varied from 2 weeks to 11 months, the average being 1.4 months. No cases of DM. (8 patients had a single abscess which responded favorably to an average of three treatments. For the other cases an average of 7.8 treatments were required for a favorable response (undefined). Effects of therapy-reduces pain and aborts the disease. No recurrences noted in 3 month follow-up., negating need for future surgery. 7895 Via Belfiore #4, San Diego, CA 92129 - Ph: (858) 901-4747 Email: [email protected] - http://www.hs-foundation.org 54