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39.1 C H A P T E R 39 Pompholyx Carlo M. Gelmetti Department of Anesthesia, Intensive Care and Dermatologic Sciences, Università degli Studi di Milano, Milan, Italy Definition. Pompholyx, also known as vesicular eczema of palms and soles, dyshidrotic eczema or dyshidrosis, is a recurring acute eczematous eruption, composed of discrete well-visible crops of vesicles situated mostly on the palms and soles, and along the sides of fingers and toes [1,2]. When pompholyx occurs on the palmar surfaces or along the sides of fingers, it may be called cheiropompholyx, and when on the soles or along the sides of toes, podopompholyx. For the former, the term ‘acute and recurrent vesicular hand dermatitis’ has been recently suggested [3]. History. Pompholyx is a Greek term meaning ‘blister ’. The disease was studied systematically only in the last century, when microscopic and bacteriological investigations became available. Fox [4] described the disease in 1873, and in 1876 Hutchinson [5] introduced the terms cheiropompholyx, podopompholyx and cheiropodopompholyx. Although histological studies demonstrated that the lesion of pompholyx was not a result of eccrine duct involvement, the debate on the aetiology of pompholyx has continued. The hypothesis of a bacterial origin, originally evoked by Unna, has not been confirmed. However, the concept of the ‘internal’ origin of pompholyx continues to appear in the dermatological literature [6]. Aetiology and pathogenesis. The cause of pompholyx is unknown. Pompholyx is often worse in warm weather and, as the synonym ‘dyshidrotic eczema’ denotes, it was thought to result from excessive sweating. It is now recognized that the term ‘dyshidrotic eczema’ is a misnomer because the disorder is not related to secretion or excretion of sweat. The intraepidermal part of the eccrine sweat duct (acrosyringium) is not the focus of the spongiotic process that characterizes the disorder. Pompholyx has also been attributed to emotional lability [7]. Although this hypothesis is unproven, many patients testify to a relationship between emotional stress and exacerbation of the disease. In one study, children Harper’s Textbook of Pediatric Dermatology, 3rd edition. Edited by A. Irvine, P. Hoeger and A. Yan. © 2011 Blackwell Publishing Ltd. with pompholyx underwent psychological testing and were noted to be submissive rather than aggressive [8]. Current knowledge suggests that pompholyx is a manifestation of an atopic diathesis [9,10]. In a recent study, all the patients affected by idiopathic pompholyx were atopic [11]. In northern Italy, an investigation of 104 subjects revealed a personal or family history of atopy in 50% of cases; in this cohort, high levels of immunoglobulin E (IgE) were present in 39 patients (35%) [12]. The particular case of simultaneous pompholyx in monozygotic twins, during their separation [13], is in favour of both familial and psychological factors. Recently, a locus at chromosome 18q22.1–18q22.3 has been idenfified in a large Chinese family affected by a rare autosomal dominant form of pompholyx [14]. A number of recent investigators have come to believe that pompholyx is an expression of allergy to nickel [15], chromium [16] and cobalt [17]. Pompholyx can be induced in nickel-sensitive subjects by oral administration of high doses of nickel [18], so pompholyx in the infant could possibly be induced by metals present in mother ’s milk [16]. In another study, a patient allergic to nickel developed pompholyx after implantation of a pacemaker that contained nickel [19]. Although pompholyx is thought by some authors to be the most common manifestation of nickel allergy, this association is unlikely to account for most cases of pompholyx in children. Other primary irritants, such as soluble oils used by metal workers [18] and direct contact allergens (primula, paraphenylenediamine, benzoisothiazolones, dichromates [19], perfumes, fragrances and balsams [20]), can also cause pompholyx, but almost always these reports have been in adults. However, glue components, chrome salts, dyes and rubber present in shoes can cause contact allergy and also provoke pompholyx in children and adolescents [21]. In some children, pompholyx may occur as an -id reaction to dermatophyte or Candida infection on the feet or hands. Usually, in children who may be wearing occlusive footwear, the mycotic infection is on the feet (tinea pedis) and the -id reaction on the hands. Bacterial and viral infections (HIV included) [22] can also provoke similar reactions. Finally, pompholyx can be induced by such medications as antiseptics, antimycotics, neomycin, 资料来自互联网,仅供科研和教学使用,使用者请于24小时内自行删除 39.2 Chapter 39 aspirin [23], methotrexate [24], mycophenolate mofetil [25] and immunoglobulins [26]. Studies of leukotrienes have offered some evidence that the pathological changes in pompholyx can be induced by these potent inflammatory mediators [12]. Hyperhidrosis is sometimes an associated feature, and the disease often worsens in hot climates or during emotional stress. Epidemiology. Pompholyx in childhood is an infrequent condition, seen in no more than four or five per 7000 paediatric patients in 1 year in the author ’s department (C. Gelmetti, unpublished data). In the author ’s experience, the patients are of prepubertal or pubertal age; in preschool-aged children, pompholyx is rare. In adults, however, pompholyx is believed to account for about 5–20% of all cases of hand eczema [12]. Pathology. The problem of the histopathological studies in pompholyx is that it is difficult to study the early features of the disease. Limited data are available in paediatric patients. The evolving lesions of pompholyx show eczematous changes, i.e. foci of spongiosis and spongiotic vesicles in the epidermis, oedema of the papillary dermis and a superficial perivascular predominantly lymphocytic infiltrate; eosinophils can be episodically observed [27]. Psoriasiform hyperplasia and scale crusts may also appear later, whereas spongiosis and the other signs of an active inflammatory process will progressively fade. Histological features of lichen simplex chronicus may be the hallmark of chronic lesions. In complicated impetiginized lesions, histology shows intraepidermal vesiculopustules together with bacteria. Erosions and even ulcerations are another possible feature. Acrosyringia are not primarily involved, but an exhaustive histological study of serial sections of pompholyx vesicles showed that sweat ducts were often pushed aside by the tense vesicles or passed between them [28,29]. The histopathological changes of pompholyx are not specific and similar findings may be seen in allergic contact dermatitis, nummular dermatitis and -id reactions. Tinea may exhibit histopathological changes similar to those of pompholyx; however, the demonstration of fungal hyphae in sections stained by haematoxylin and eosin or by more specific stain (e.g. periodic acid–Schiff) makes the differentiation easy. Clinical features. Pompholyx is classically characterized by the sudden onset of crops of tense clear vesicles, which have been described as resembling tapioca or to be ‘sagolike’ [30,31]. It may occur at any age but is rare under the age of 10 years. The eruption, generally bilateral, can affect the sides of fingers and toes, palms and soles (Figs 39.1 and 39.2). Erythema, when present, is not a distinctive finding. Subjective symptoms are variable, but they Fig. 39.1 In this young boy, pompholyx is in its florid stage, represented by crops of tense clear vesicles in a bilateral distribution affecting the toes. Similar lesions were also present on the palms. Fig. 39.2 In this adolescent, pompholyx is in a late phase in which small crusts are intermingled with scales but always in a typical bilateral distribution. are generally present during or before the attack; children describe a sensation of pruritus or prickling. Pruritus may sometimes be severe. Vesicles, usually discrete at the beginning (Fig. 39.3), may become confluent and form large multiloculated bullae, especially on the feet (Fig. 39.4). Generally, the blisters are distributed symmetrically and quite regularly. Rarely, lesions have been observed to take arciform, annular or target-like shapes [32]. The thickness of the involved skin keeps the vesicles intact, if they are not scratched or rubbed for many days. Even in the absence of treatment, the course of the disease can vary greatly from case to case; one common occurrence is a series of attacks that last for a few days to a few weeks and subside spontaneously in 2–3 weeks with desquamation. Exacerbations are the rule, but it is difficult to predict both the number and the severity of the attacks. When superinfection of primary blisters occurs, pustular lesions may develop. Erosions and ulcerations may also complicate the clinical appearance. Extensive vesiculation may cause disability by compromising 资料来自互联网,仅供科研和教学使用,使用者请于24小时内自行删除 Pompholyx Fig. 39.3 Discrete vesicles are present on the plantar surface of this infant who is affected by atopic dermatitis. Fig. 39.4 A closer view of the lesions illustrated in Fig. 39.1 reveals that the vesicles have become confluent and have formed multiloculated bullae. the function of hands (and feet) and therefore disturbing school activities. Secondary lymphoedema [33] can occur but constitutional symptoms, such as fever, are exceptional. In some of those patients, lymphoscintigraphy revealed a failure of small lymphatics of the hand to absorb and drain lymph to regional nodes [34]. Differential diagnosis. Infantile acropustulosis characteristically affects darker-skinned infants in the first 39.3 months of life [35,36], an age at which pompholyx is exceptional, and the lesions are easily seen as pustular rather than vesicular. Histopathologically, eosinophils and neutrophils predominate. ‘Hand, foot and mouth disease’ should be easily differentiated from pompholyx by the presence of lesions on the oral mucosa. In hand, foot and mouth disease, vesicles are oval, correspond with the hand-lines, show a typical lilac border and do not tend to merge but remain individually distinct [37]. Palmoplantar psoriasis starts with pustules rather than vesicles and the subjective sensation is burning rather than itching [38]. Dermatitis herpetiformis, besides the other symptoms associated with gluten sensitivity, can involve the palmar and plantar surfaces, but the flexor surfaces of the fingers rather than their sides are affected and the blisters are often haemorrhagic [39]. Blistering distal dactylitis is a rare streptococcal disease of children [40] which, unlike pompholyx, is rarely pruritic and, rather than being present on the sides of fingers, is found typically at their tips. Localized bullous pemphigoid, another rare disease, shows blisters, and not vesicles, which are often haemorrhagic [41]. Recurrent palmoplantar hidradenitis [42] is an entity that has in common with pompholyx only the topography; the lesions are completely different as they are tender and inflamed papules and nodules. Papular–purpuric ‘gloves and socks’ syndrome, previously described in adults as a characteristic exanthem affecting the distal part of the extremities, has also been observed in children [43]; the lesions are erythematous, involving the dorsa of the hands and feet, which rapidly become purpuric. Treatment. Treatment of pompholyx should be based around avoiding the offending cause. However, this is difficult, especially in children, in whom the possibility of pompholyx from nickel allergy or superficial mycosis is low. Causal treatment of the pompholyx related to metal allergy includes avoidance of any contact with nickel-containing metals, a low-nickel/chromium/cobalt diet (in both the child and the mother in cases of breastfed infants) [16,17] and, in cases of clearly defined nickel allergy, the administration of chelating agents, such as diethyldithiocarbamate and tetraethylthiuram disulphide [44] and disodium cromoglycate [45] have occasionally been helpful. Causal treatment of pompholyx related to superficial mycosis involves the eradication of the fungus and local hygiene. Treatment of pompholyx associated with atopy, the most common form in children, involves symptomatic treatment, including topical corticosteroids of medium or high potency [46], zinc oxide ointments and oral antihistamines. Topical steroids are used widely for management of the condition, but their efficacy is limited by low 资料来自互联网,仅供科研和教学使用,使用者请于24小时内自行删除 39.4 Chapter 39 percutaneous absorption of drugs in palmar–plantar areas unless super-potent molecules are used. Zinc oxide or water pastes can be used in the acute phase for their mild anti-inflammatory and protective properties. Oral antihistamines appear, in most cases, only moderately effective. A short course of oral steroids is rarely indicated but it may be justified in severe cases. In adult patients, the following treatments have also been used: intradermal botulinum toxin [47], topical calcineurin inhibitors: tacrolimus [48] and pimecrolimus [49], radiation therapy [50], azathioprine [51], methotrexate [52], mycophenolate mofetil [53], UVA1 irradiation, topical or bath PUVA [54] and tap water iontophoresis with pulsed direct current [55]. Management of pompholyx also includes in the acute stage, soaks or wet dressings to cleanse the affected area and to relieve discomfort, such as potassium permanganate at a dilution of 1 : 10,000. Large bullae can cause discomfort or even pain and may be aspirated using a sterile syringe. Systemic antibiotics should not be used unless impetiginization supervenes. Secondary bacterial infection is most likely caused by staphylococci, so an appropriate antibiotic should be prescribed. Harsh detergents should be avoided and the hands should be protected routinely with cotton gloves; some patients complain of sudden recurrences even when touching certain foods, such as citrus fruits or tomatoes. Prophylactic measures should be constantly observed, especially in school (e.g. the use of alcohol-based marker pens must be discouraged). As the acute phase subsides, the wet dressing or soaks should be discontinued and replaced with protective topical treatments or with tar preparations; the latter are indicated for those cases of chronic pompholyx that have entered the hyperkeratotic phase. Experiences relating to pompholyx with bexarotene gel, topical kellin and natural sunlight [56] have been more limited. New types of anti-inflammatory oral drugs such as leukotriene inhibitors, phosphodiesterase inhibitors and alitretinoin are under study as alternatives to ultraviolet phototherapy [46]. References 1 Lofgren SM, Warshaw EM. Dyshidrosis: epidemiology, clinical characteristics, and therapy. Dermatitis 2006;17:165–81. 2 Vocks E, Plotz SG, Ring J. The Dyshidrotic Eczema Area and Severity Index: a score developed for the assessment of dyshidrotic eczema. Dermatology 1999;198:265–9. 3 Storrs FJ. Acute and recurrent vesicular hand dermatitis not pompholyx or dyshidrosis. Arch Dermatol 2007;143:1578–80. 4 Fox T. Skin Diseases: Their Description, Pathology, Diagnosis, and Treatment, 3rd edn. London: Henry Renshaw, 1873:476. 5 Hutchinson J. Cheiropompholyx. Lancet 1876;i:618–9. 6 Erdmann SM, Werfel T. [Hematogenous contact eczema induced by foods.] Hautarzt 2006;57:116–20. 7 Kellem RE. Dyshidrotic hand eczema: a psychotherapeutic approach. Cutis 1975;16:875–8. 8 Hansen O, Kucheler T, Lotz GR et al. My fingers itch, but my hands are bound: an exploratory psychosomatic study of patients with dyshidrosis of the hands. J Psychosom Med Psychoanal 1981;27:275–90. 9 Norris PG, Levene GM. Pompholyx occurring during hospital admission for treatment of atopic dermatitis. Clin Exp Dermatol 1988;12:189–90. 10 Schwanitz HJ. Atopic Palmoplantar Eczema. Berlin: Springer, 1988. 11 Guillet MH, Wierzbicka E, Guillet S, Dagregorio G, Guillet G. A 3-year causative study of pompholyx in 120 patients. Arch Dermatol 2007;143:1504–8. 12 Lodi A, Betti R, Chiarelli G et al. Epidemiological, clinical and allergological observations on pompholyx. Contact Dermatitis 1992;26: 17–21. 13 Lorincz AL, Grauer FH. Simultaneous dyshidrosis in monozygotic twins during their separation. Arch Dermatol 1956;74:245–52. 14 Chen JJ, Liang YH, Zhou FS et al. The gene for a rare autosomal dominant form of pompholyx maps to chromosome 18q22.1–18q22.3. J Invest Dermatol 2006;126:300–4. 15 Fowler JF Jr, Storrs FJ. Nickel allergy and dyshidrotic eczema: are they related? Am J Contact Dermatitis 2001;12:119–21. 16 Adachi A, Horikawa T. [Pompholyx of the infant possibly induced by systemic metal allergy to chromium in mother ’s milk.] Arerugi 2007;56:703–8. 17 Stuckert J, Nedorost S. Low-cobalt diet for dyshidrotic eczema patients. Contact Dermatitis 2008;59:361–5. 18 Prystupa K, Rudzki E. Oral tolerance of nickel in patients with dyshidrosis. Contact Dermatitis 2000;42:276–7. 19 Landwehr AJ, van-Ketel WG. Pompholyx after implantation of a nickel-containing pacemaker in a nickel-allergic patient. Contact Dermatitis 1983;9:147. 20 Jain VK, Aggarwal K, Passi S, Gupta S. Role of contact allergens in pompholyx. J Dermatol 2004;31:188–93. 21 Pitche P, Boukari M, Tchangai-Walla K. Factors associated with palmoplantar or plantar pompholyx: a case–control study. Ann Dermatol Venereol 2006;133:139–43. 22 MacConnachie AA, Smith CC. Pompholyx eczema as a manifestation of HIV infection: response to antiretroviral therapy. Acta Derm Venereol 2007;87:378–9. 23 Edman B. Palmar eczema: a pathogenic role for acetylsalicylic acid, contraceptives and smoking? Acta Dermatol Venereol 1988;68:402–7. 24 Martins da Cunha AC, Rappersberger K, Gadner H. Toxic skin reaction restricted to palms and soles after high dose methotrexate. Pediatr Hematol Oncol 1991;8:277–80. 25 Semhoun-Ducloux S, Ducloux D, Miguet JP. Mycophenolate mofetilinduced dyshidrotic eczema. Ann Intern Med 2000;132:417. 26 Rhee DY, Park GH, Chang SE et al. Pompholyx after intravenous immunoglobulin therapy for treatment of Guillain–Barré syndrome. J Eur Acad Dermatol Venereol 2009;23:602–4. 27 Caputo R, Ackermann AB, Sison-Torre E. Dyshidrotic dermatitis. In: Pediatric Dermatology and Dermatopathology, Vol. 2. Philadelphia: Lea & Febiger, 1993:119–29. 28 Kuntzer H, Wurzel RM, Wolff HH. Are acrosyringia involved in the pathogenesis of ‘dyshidrosis’? Am J Dermatopathol 1986;8:109–16. 29 Simons RDGP. Eczema of the Hands, 2nd edn. Basle: Karger, 1966. 30 Bikowski JB. Hand eczema: diagnosis and management. Cutis 2008;82(Suppl):9–15. 31 Weston WL, Lane AT, Morelli JG. Color Textbook of Pediatric Dermatology. St Louis: Mosby, 2007. 32 Batres E. Annular pompholyx. Cutis 1979;23:819–21. 33 Gach, JE, King CM. Constitutional pompholyx eczema complicated by secondary lymphoedema. Acta Dermatol Venereol 2001;81:437–8. 34 Pearce VJ, Mortimer PS. Hand dermatitis and lymphoedema. Br J Dermatol 2009;161:177–80. 35 Mazereeuw-Hautier J. [Infantile acropustulosis.] Presse Med 2004; 33:1352–4. 资料来自互联网,仅供科研和教学使用,使用者请于24小时内自行删除 Pompholyx 36 Braun-Falco M, Stachowitz S, Schnopp C et al. Infantile acropustulosis successfully controlled with topical corticosteroids under damp tubular retention bandages. Acta Derm Venereol 2001;81: 140–1. 37 Tai WC, Hsieh HJ, Wu MT. Hand, foot and mouth disease in a healthy adult caused by intrafamilial transmission of enterovirus 71. Br J Dermatol 2009;160:890–2. 38 Janniger CK, Schwartz RA, Musumeci ML et al. Infantile psoriasis. Cutis 2005;76:173–7. 39 Caproni M, Antiga E, Melani L et al. Guidelines for the diagnosis and treatment of dermatitis herpetiformis. J Eur Acad Dermatol Venereol 2009;23:633–8. 40 Scheinfeld NS. Is blistering distal dactylitis a variant of bullous impetigo? Clin Exp Dermatol 2007;32:314–6. 41 Gajic-Veljic M, Nikolic M, Medenica L. Juvenile bullous pemphigoid: the presentation and follow-up of six cases. J Eur Acad Dermatol Venereol 2010;24:69–72. 42 Kluger N, Moguelet P, Khosrotehrani K et al. Idiopathic recurrent palmoplantar hidradenitis: a case with late onset and long-lasting course. Clin Exp Dermatol 2007;32:217–8. 43 Fretzayas A, Douros K, Moustaki M, Nicolaidou P. Papular-purpuric gloves and socks syndrome in children and adolescents. Pediatr Infect Dis J 2009;28:250–2. 44 Menne T, Kaaber K. Treatment of pompholyx due to nickel allergy with chelating agents. Contact Dermatitis 1978;4:289–90. 45 Pigatto PD, Gibelli E, Fumagalli M et al. Disodium cromoglycate versus diet in the treatment and prevention of nickel-positive pompholyx. Contact Dermatitis 1990;22:27–31. 46 Wollina U. Pompholyx: what’s new? Expert Opin Investig Drugs 2008;17:897–904. 39.5 47 Kontochristopoulos G, Gregoriou S, Agiasofitou E et al. Regression of relapsing dyshidrotic eczema after treatment of concomitant hyperhidrosis with botulinum toxin-A. Dermatol Surg 2007;33:1289–90. 48 Schnopp C, Remling R, Mohrenschlager M et al. Topical tacrolimus (FK506) and mometasone furoate in treatment of dyshidrotic palmar eczema: a randomized, observer-blinded trial. J Am Acad Dermatol 2002;46:73–7. 49 Schurmeyer-Horst F, Luger TA, Bohm M. Long-term efficacy of occlusive therapy with topical pimecrolimus in severe dyshidrosiform hand and foot eczema. Dermatology 2007;214:99–100. 50 Stambaugh MD, DeNittis AS, Wallner P et al. Complete remission of refractory dyshidrotic eczema with the use of radiation therapy. Cutis 2000;65:211–4. 51 Scerri L. Azathioprine in dermatological practice: an overview with special emphasis on its use in non-bullous inflammatory dermatoses. Adv Exp Med Biol 1999;455:343–8. 52 Egan CA, Rallis TM, Meadows KP et al. Low-dose oral methotrexate treatment for recalcitrant palmoplantar pompholyx. J Am Acad Dermatol 1999;40:612–4. 53 Pickenacker A, Luger TA, Schwarz T. Dyshidrotic eczema treated with mycophenolate mofetil. Arch Dermatol 1998;134:378–9. 54 Tzaneva S, Kittler H, Thallinger C et al. Oral vs. bath PUVA using 8-methoxypsoralen for chronic palmoplantar eczema. Photodermatol Photoimmunol Photomed 2009;25:101–5. 55 Odia S, Vocks E, Rakoski J et al. Successful treatment of dyshidrotic hand eczema using tap water iontophoresis with pulsed direct current. Acta Dermatol Venereol 1996;76:472–4. 56 Capella GL. Topical khellin and natural sunlight in the outpatient treatment of recalcitrant palmoplantar pompholyx: report of an open pilot study. Dermatology 2005;211:381–3. 资料来自互联网,仅供科研和教学使用,使用者请于24小时内自行删除