Download Pompholyx

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,
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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
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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
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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.
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