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Challenging Cases in Dermatology: The Itchy, Painful or Burning Vulva Ginat W. Mirowski, DMD, MD Department of Oral Pathology, Medicine, Radiology Department of Dermatology Indiana University Full Disclosure of Faculty Financial Interests or Relationships I agree to follow the UIC and ACCME policies and declare that I do not have a financial interest or other relationship with any manufacturers of any commercial products that may be discussed during this presentation. 1 Conflict of Interest • I have no relevant conflicts of interest • I will discuss use of off label medications • The medications and treatment suggestions mentioned in this talk are based on my current use patterns; other treatment options may be available Vulvar Dermatology Objectives • Present a dermatologist’s approach to the patient with vulvar complaints • Itch • Pain • Burning • Share clinical pearls for diagnosis • Discuss systemic considerations 2 Vulvar Dermatology Therapeutic Objectives • Review treatment pearls related to vulvar dermatology • Discuss treatment regimens • topical regimens • systemic regimens • supportive care • Underscore potential hazards and obstacles to maximize therapeutic success Have confidence!! • Vulvar diseases can be challenging • Vulvar patients can be frustrated • Treatment of vulvar disease may be challenging • But…success is possible and very much achievable 3 Evaluation of Patient with Vulvar Concerns • Identify potential etiologies and exacerbating factors • Medical history and review of systems • Identify extent of involvement and impact • Directed physical examination skin and mucous membranes • Treat and eliminate confounding conditions • Remove all potential local irritants Pruritus Vulva (partial list of dermatologic and infectious causes) • • • • • • • • • Eczematous dermatitis Lichen sclerosus Lichen planus Psoriasis Contact dermatitis Dermatophyte infection Paget’s disease Syringomas Squamous cell carcinoma • Candidiasis (erythematous and pseudomembranous) • Beta strep • Secondary syphilis • Pityriasis rosea • Discoid lupus erythematosus • Diabetes • Hepatic failure • Iron deficiency anemia • Hypothyroidism 4 Vulvar Examination Pearls and Pitfalls • • • • • • • Clinician and patient à comfortable Engage patient with eye contact & hand mirror Moisture alters the appearance Gently pat with gauze to dry off the mucosa Minor trauma alters the primary morphology Limited number of reaction patterns Histology is not specific but often helpful Complete Mucocutaneous Exam* • Skin, hair, nails • Oral and ocular mucous membranes inc conjunctiva, esophagus, larynx, ears • Vulvar exam • Vaginal exam with speculum; wet mount • Patient participation * often requires consultation referral 5 Start at the Top! Work to Inside! The Normal Vulva • • • • • • • • • • • Abdomen, thighs Mons or mons pubis Clitoral hood, clitoris Labia majora or outer labia Interlabial sulcus Labia minora or inner labia Vaginal introitus/vestibule Posterior fourchette Perineal body/perineum Perianal area, buttocks Gluteal cleft Illustrated by Dawn Danby and Paul Waggoner Challenging Vulvar Cases: Pruritic, Painful or Burning Vulva • Primary or essential à no primary etiology identified • Secondary à infections, dermatoses, systemic diseases, malignancy, or neurologic conditions • Multifactorial or mixed Primary Secondary (essential) Mixed 6 Eczematous (Vulvar) Dermatitis • External sensation of itching • Need to scratch or rub • Skin thickens à prominent skin fold markings (lichenification) • Excoriations /pigmentary changes • Psychologically distressing • Socially embarrassing Eczematous Dermatitis Clinical Features • Poorly demarcated plaques • Variable erosions excoriations erythema pigmentary change • Severe pruritus • Lichenification 7 Eczematous Vulvar Dermatitis Aggravating Factors • Body fluids (sweat, urine, feces) • Excessive bathing (soaps, detergents, bubble baths, douche) • Tampons/pads • Condoms/spermicidal agents • • • • Feminine hygiene products Lubricants Toilet paper Medications • antifungals/antibiotics • corticosteroids • hormones • others Candidiasis Clinical pearls • Common in both children and adults • Erythematous plaques • Satellite papules • Edema of labia • +/- discharge • Intertrigo - erosive 8 Evaluation: KOH Microscopy • Confirm at bedside • Pseudohyphae – Doubly refractile – Tapering at ends – Branching • Budding yeast – bowling pins Challenging Unknown: Red Itchy Vulva 9 Cutaneous Psoriasis • 2-5% in general population • Sharply demarcated erythematous papules and plaques with silvery scale • Symptoms: absent to severe pruritus • Findings: Scalp, elbows, and knees > Intergluteal cleft pinking • Rarely entire skin surface is affected • Nail involvement ~ 50% of patients Psoriasis • Papulosquamous eruption; joint involvement • May occur at any age; onset is usually gradual • Cellular turnover is increased sevenfold and decreased from the normal 28 days to 3 or 4 days • + family history in 1/3; genetic predisposition • Precipitated by infection, trauma, or stress • Exacerbated by beta blockers and lithium 10 Vulvar Psoriasis • Well-circumscribed • Pink plaques • Little to no scale • Inguinal crease, intergluteal fold, mons pubis, labia majora, perineum • Symptoms vary from absent to severe pruritus Vulvar Psoriasis • Pink patches • No satellite lesions • D/dx: Candidiasis, intertrigo, seborrheic dermatitis • Secondarily infected candida, strep 11 Candidiasis vs Psoriasis • • • • Common Erythematous plaques Satellite papules Extra vaginal, Intertrigo – erosive, edema • Rare in children • Erythematous patches • Extravaginal • Intertrigo • Gluteal pinking Dermatophytosis “Jock itch” or Tinea Cruris • Papulosquamous eruption • Infection • Ring like plaques with central clearing • Border - fine scale • Symptoms variable • KOH positive 12 Dermatophyte KOH Preparation Unknown Vulvar Pruritus • Unresponsive • Topical anti-yeast medications • Topical steroids helped slightly • Protopic, Elidel very irritating • Diagnostic biopsy was performed 13 Lichen Planus • Chronic pruritic mucocutaneous dermatosis • Polygonal purple papules, flat topped • Wrists and ankles > mucous membranes • Unknown etiology • A cellular immune mechanism is suspected • Activated T-cells seen in early lesionsà Target antigenically altered basal cells • Suppressor T-cells predominate in older lesions Lichen Planus Incidence • • • • • • In US 0.44% In dermatology clinics 1.5% Oral medicine clinics 5% No such number in GYN clinic Oral disease 1/2000 Genital involvement 1/4000 – 1/8000 14 Cutaneous Lichen Planus Pruritic polygonal flat topped purple or pink papules Oral Lichen Planus • Reticulated white plaque (net-like) • Buccal mucosa > gingivae or tongue • Usually asymptomatic • Erosive à painful • D/Dx: LP, PV, BP • Biopsy diagnostic 15 Genital Lichen Planus • • • • Women >> men Waxing & waning Decades Mistaken for recurrent “Candida” or “Herpes” • 5th - 6th decade, all ages Vulvovaginal Lichen Planus • White, lacey papules (reticulated plaque) • Erosion 16 Erosive Vulvar Lichen Planus Prospective study n = 114 women x 5 yrs Presenting signs • Erosions (90%) • White reticulations (82%) (may be asymptomatic) • Hypertrophic changes (20-25%) Presenting symptoms • Pain (80%) • Pruritus (65%) • Dyspareunia (61%) • Irritation (48%) Cooper and Wojnarowska, Arch Derm 2006 Vulvovaginal Scarring in Lichen Planus • Burying of the clitoris • Loss of the right labium minus • Agglutination/ resorption of the labium minora • Vaginal adhesions and stenosis • Pink perirectal papules Bethanee Schlosser 17 Vulvovaginal Gingival Syndrome Vulvar Scarring Loss of labia minora Burring of clitoris Vaginal scarring Oral and gingiva findings Common May precede, follow or occur concurrently Esophageal Lichen Planus • • • • • Review 72 cases 7:1 women : men Median age 62 years Dysphagia 81% Odynophagia 24% • • • • Oral LP 89% Vulvar /anal LP 42% Skin LP 38% Esophageal disease • Proximal 64% • Distal 11% • Both 26% Fox, Lightdale, Grossman JAAD 2011; 65:175-83 18 Lichen Planus and Cicatricial Conjunctivitis • Indistinguishable from other forms of cicatricial conjunctivitis • 11 new and 18 published • All had other mucosal sites • D/dx: pemphigoid, Stevens Johnson, Atopic Keratoconjunctivitis • Tx: corticosteroid or cyclosporine drops Brewer et al. JEADV 2011 25(1):100-4 Lichen Planus Histology • Hyperkeratosis • Acanthosis • Dyskeratosis • Intense band-like inflammatory infiltrate • Hypergranulosis • Basal cell liquefaction and destruction • Immunofluorescence + 19 Vulvar Lichen Planus Clinical Features • Think of lichenoid mucositis secondary to medications • NSAIA • Antihypertensive agents • Long list of other Lichenoid Drug Reactions (Partial List) • • • • • • • • • • • Allopurinol Arsenicals Aspirin Bismuth Carbamazepine Chloroquine Chlorothiazide Chlorpropamide Dapsone Furosemide Gold salts • • • • • • • • • • • • Hydroxychloroquine Imipramine Interferons Levamisole Lithium Mercury Methyldopa Naproxen Palladium Para-aminosalicylic acid Penicillamine Phenothiazine • • • • • • • • • • • Phenytoin Procainamide Propranolol Quinacrine Quinidine Spironolactone Streptomycin Tetracycline Tolbutamide Triprolidine Zidovudine 20 Challenging Case • Patient know to have Lichen Planus • Presents for routine follow-up • She reports all is going well and she is asymptomatic • But… not all is well Vulvar SCC and LP • Occasionally reported • Incidence is rare • 10 of 145 patients had a genital malignant neoplasm or a history of the same Cooper, Haefner and AbrahamsGessel 2008 Archives of Dermatology 21 Challenging Unknown • Sharply circumscribed plaques • Variable color pink, brown, blue, white • D/dx: SCC/bowens disease, wart, lichen simplex chronicus, psoriasis, lichen planus, discoid lupus Unknown Other mucocutaneous findings 22 Discoid Lupus Erythematosus • Sharply circumscribed plaques • Variable color pink, brown, blue, white • Sun exposed areas commonly involved • When below the waist, consider systemic disease (ANA) Vesiculobullous Diseases Definition • Rare, inherited or acquired diseases • Characterized by blisters and ulcers • Mucous membranes, skin and systemic involvement • Primary lesions --> vesicles and bullae • Secondary lesions --> erythema, erosions, ulcers, adhesions, atrophy and scaring 23 Vesiculobullous and Erosive Diseases • Lichen planus • Cicatricial pemphigoid (benign mucous membrane pemphigoid • Lupus erythematosus • Herpes simplex virus • Pemphigus vulgaris • Erythema multiforme • Aphthous ulcers • Behcet’s syndrome Cicatricial Pemphigoid • Autoimmune condition • Tense bullae or erosions • Cutaneous and mucosal involvement oral, vulvar, conjunctiva 24 Pemphigus Vulgaris • Rare autoimmune condition • Uniformly fatal prior to advent of steroids • Oral involvement early • Erosions >> bullae Pemphigus Vulgaris Cutaneous Manifestations • Flaccid bullae • Intraepithelial acantholysis • Follow serum ab levels • Ab reflect disease activity in patients 25 Pemphigus Vulgaris Pathologic Findings Atrophic Conditions • • • • Candidiasis Lichen sclerosus Post steroid atrophy Estrogen deficiency 26 Erythematous Candidiasis • Erythema and erosions • Edema • Satellite papules may be hard to find • Discharge is gray • KOH may not be diagnostic Vulvar Lichen sclerosus • • • • • • Chronic inflammatory dermatosis Waxing and waning course Hallopeau in 1887 Wide clinical spectrum Pruritus common Bimodal distribution (women>girls, men) • Unknown etiology 27 Lichen Sclerosus: Clinical Features • White, crinkled lesions; Often hemorrhagic and eroded • Located on the modified mucous membranes: vulva, perineum and perianal • No vaginal involvement • Skin involvement is not uncommon Vulvar Lichen Sclerosus: Clinical Features • Hypopigmented atrophic macules or patches; Scarring is common • Genital disease à hemorrhage and erosions ; May be mistaken for child or elder abuse • "figure eight" • Skin lesions in 20% • NO vaginal involvement *** • Rare oral cases • Autoimmune (thyroid, vitiligo, pernicious anemia) Cooper et al. Arch Dermatol. 2008;144(11):1432-143 28 Histology of Vulva Lichen Sclerosus • Epithelium is normal • Submucosa shows band of hyalinization of the underlying dermis • (appears structureless, edematous with few cells) • Slight inflammatory band Lichen Sclerosus and Increased Risk of Malignancy • • • • • SCC<5%; verrucous carcinoma case reports Vulvar SCC ~ 60% occur on LS background Even when LS is successfully treated Life long annual examinations Biopsy of any nonhealing erosions or papules that appear • http://www.niams.nih.gov/Health_Info/ Jones et al. Am J Obstet Gynecol 2008: 198:496 Lichen_Sclerosus/default.asp Walkden J Obstet Gynaecol 1997; 17:551–3. 29 Approach to the Treatment Of Vulvar Disorders • Obtain a definitive diagnosis • Culture (viral, bacterial, fungal) • Biopsy for routine H&E and IF • Give yourself and the patient time • Remain non-judgmental and supportive • Avoid telephone diagnoses • Reexamine patient; Obtain cultures; Biopsy • Treat one condition at a time (Osler) Papulosquamous and Eczematous Disorders • Corticosteroids first line treatment Topical vs. Systemic Start with super-potent and taper q 2 weeks bid --> qd --> MWF • Steroid sparing agents • Symptomatic measures • Prophylaxis for secondary infections Candida, Dermatophyte, Virus 30 Steroid Complications Stria HSV Atrophy Striae (stretch marks) Telangiectasias Secondary infections Dermatitis Topical Steroids Important Issues to Consider • Potency of agent • Vehicle • Affected areas • Non-erosive disease on skin --> cream - base • Mucosal disease of vulva --> ointment - base • Vulvovaginal disease --> applicator, foam, suppositories • Oral disease --> gels or elixir • Consider intralesional steroids 31 Supportive Aspect of Treatment of Vulvar Disorders • Address urinary and fecal incontinence • Barrier petrolatum and zinc based ointments • Use systemic medications whenever possible • Treat low estrogen states (postpartum, peri and post menopause) with estrogen to restore vaginal and vulvar mucosal barrier function Vulvar Pruritus • Break the scratch-itch cycle • Non-sedating antihistamines in morning • Sedating antihistamines at bedtime • Hydroxyzine 10-75mg • Doxepin 10-30mg • Cool compresses, frozen peas (wrapped in a towel) 32 Summary of Therapeutic Tips • Ointments are preferred over creams • Vulvar disease can be relatively steroid-resistant • Use ultra potent steroids • Monitor for atrophy at crural crease, thighs, buttocks • Limit topicals • Use systemic antibiotics, antifungals when available Multidisciplinary Team • Nurse practitioner • Physician assistant • PCP • Gynecologist • Dentist • Ophthalmologist • Gastroenterologist • Urologist • Psychologist • Sexual therapist • Physical therapist • Marriage counselor • Others Andrew Luck 33 Vulvar Dermatology • You are not alone • Many patients believe that the vulvar dermatitis can be a devil of a problem • Now you have the tools to treat it References • Genital Dermatology Atlas by Dr. Libby Edwards (2010) • Obstetric and Gynecologic Dermatology by Black et al. (2008) • Seminars in Cutaneous Medicine and Surgery. WB Saunders, September (1998) • Dermatologic Clinics: Vulvovaginal Dermatology edited by Dr. Libby Edwards (2010) • WWW.ISSVD.ORG 34 Where to Biopsy • Histology (H&E) • Optimal site is an active lesion or if ulcerated at the border (normal and abnormal) • Immunofluorescence • Optimal site is perilesional (normal) 35 Immunofluorescence • • • • • Laboratory technique Demonstrates the presence of autoantibodies Tissues (direct) or serum (indirect) Normal tissue used as a substrate Different substrates for specific diseases General Therapeutic Approach Vulvar Inflammatory Conditions • Eliminate contact with all potential irritants • Avoid any chemical or product that could confound treatment • Use systemic regimens if available • Start low and go slow • Consider supportive care 36 Avoid Topical Agents • • • • Toilet paper Lotions / creams Disposable wipes Feminine deodorant products / douches • Sanitary napkins • Tampons • Pantyhose and girdles • • • • • • • • Suppositories Soaps Detergents Baths Bubble bath / bath salts Condoms Spermicidal agents Lubricants What Is Safe? • • • • Water • One’s hands • Cotton towels / diapers • Cotton underwear Pat dry, do not rub Dry with a blow-drier on cool Systemic agents whenever possible 37 Lubricants • • • • • • Retard evaporation of water Replace lipids +/Vehicles for medications Limit friction Help with reepithelialization and wound healing Smooth over scale and roughened surfaces Vulvar Vestibulitis • Burning, irritation, rawness • Limited to vulvar vestibule • Elicited by physical touch or pressure • painful foreplay • using tampons • wearing blue jeans • vaginal penetration • wiping with toilet paper • riding bicycle 38 Severity of Vulvar Vestibulitis • Grade I • Grade II • Grade III Causes discomfort but does not prevent intercourse Frequently prevents sexual intercourse Completely prevents sexual sexual intercourse Marinoff and Turner, Dermatol Clin, 1992 Pain Medications Tricyclic Antidepressants • Amitriptyline (Elavil ®) (10mg, 25mg, 75mg) • Begin at 1/2 a tab qHS and increase slowly as tolerated to a maximum dose of 75 mg • Limit drowsiness by taking it early in evening • Xerostomia and xerophthalmia • Appetite stimulation • Desipramine (Norpramin ®) (25mg,100mg) • Less sedating • Anxiety and jitteriness • Cardiac arrhythmias 39 Pain Medications Anticonvulsant • Gabapentin (Neurontin®) (100mg, 300mg, 400mg) • 300mg TID • Dizziness, somnolence, fatigue, ataxia • Pancreatic acinar CA, a rare tumor, was seen in male rats • Negative Ames test Pitfalls of Treatment • Progressive scarring • Secondary infection • Candidiasis – common given use of steroids • Herpes – reactivation • Postinflammatory hyperpigmentation 40
