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Peeling skin syndrome

European Journal of Dermatology. Volume 16, Number 3, 287-9, May-June 2006, Clinical report

Summary

Author(s) : Turna İlknur, Melda Demirtaşoğlu, Sevgi Akarsu, Banu Lebe, Ali Tahsin Güneş, Şebnem Özkan , Dept of Dermatology Dokuz Eylül University, Faculty of Medicine, İzmir, Turkey, Department of Pathology, Eylül University, Faculty of Medicine, İzmir, Turkey.

Summary : Peeling skin syndrome is a rare disease characterized by widespread painless peeling of the skin. To date, several cases have been described with different clinical features called peeling skin syndrome. Previous reports describe two types (type A and type B) of peeling skin syndrome, both of which show generalized desquamation, sparing palms and soles. We report a 23-year old man who has been classified as neither type A nor type B, and whose history, clinical features and histopathological findings led to a diagnosis of peeling skin syndrome. In addition, the desquamation pattern in our patient was different from that of both types because our case’s palms and soles were involved too.

Keywords : deciduous skin, keratolysis exfoliativa congenital, peeling skin syndrome

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ARTICLE

Auteur(s) : Turna İlknur¹, Melda Demirtaşoğlu¹, Sevgi Akarsu¹, Banu Lebe², Ali Tahsin Güneş¹, Şebnem Özkan¹

¹Dept of Dermatology Dokuz Eylül University, Faculty of Medicine, İzmir, Turkey
²Department of Pathology, Eylül University, Faculty of Medicine, İzmir, Turkey

Peeling skin syndrome is a rare and poorly classified genodermatosis with a different clinical picture, and is mainly characterized by spontaneous separation of the stratum corneum [1-3]. Histologically, the separation is intracorneal or subcorneal in the peeling skin syndrome [1, 2]. This syndrome can be sporadic or familial, and it has no ethnic predominance [4]. The onset of symptoms is usually at birth or shortly thereafter, but it has also reported that the onset may occur during adulthood too [1, 5]. Although the patients have shedding of the skin, they are otherwise asymptomatic and in good general health [3]. To date, no effective treatment for peeling skin syndrome has been reported. Treatment with topical corticosteroids, UVB, methotrexate, etretinate, isotretinoin was found to be ineffective [6].Due to its clinical heterogeneity, the disorder has recently been classified into two types (A and B) depending on clinical, biological, and pathologic criteria [2, 4]. However, the clinical features of our case showed that neither type was sufficient for the thorough definition of the clinical spectrum of peeling skin syndrome.

Case report

A 23-year-old white man was admitted for the evaluation of spontaneous peeling of his skin, which had started three days before his admission. The patient said that the peeling had occurred episodically since he was 11-years-old, and that if he happened to peel off sheets of the skin, he had no pain. These episodes occurred three or four times every year. Each episode of shedding was reported to last 20-30 days. Between these episodes, the patient was completely normal, and had no residual erythematous or pigmentary skin lesions. The patient noted that the remission time between the episodes was shorter in winter than in summer. The patient observed an erythema period that lasted 1-3 days before each peeling episode. He also described mild pruritus during the skin shedding episodes which were always preceded by mild fever, malaise, and weakness. There was no history of blistering of the skin. He had used several topical agents without benefit. The disorder showed no improvement through the years. He had no history of drug consumption or any other factors responsible for precipitating or aggravating the shedding episodes. He was the product of a full-term and uncomplicated pregnancy by vaginal delivery. Neither his 17-year-old sister nor other family members had similar complaints.

Clinical examination revealed that peeling of the skin started initially from hands and feet. The extremities and face were involved on the sixth or seventh day and the trunk was affected at the end of the eleventh day. Before peelings developed, erythema occurred on the skin for 2-3 days, and it resolved with the beginning of exfoliation. The exfoliation occurred spontaneously and the present skin shedding episode lasted for twenty days. While the sheets peeled off from hands and feet were large and thick (figure 1), scales on the other body regions were small and delicate (figure 2). His general health was good during the peeling episode and we did not find any fever during that period. The mucous membranes and hairs remained normal during the peeling episode. Discrete blisters had never been present. Some fingernails had distal onycholysis.

The patient’s complete blood count, erythrocyte sedimentation rate, routine blood chemistry, urinalysis, serum ceruloplasmin, copper, iron, iron-binding capacity, IgE level were within normal limits. Plasma and urinary amino acid analysis did not show deviation. Mycological examination of the nail was negative.

Histopathological examination of the skin specimen from the back of the leg showed subcorneal separation with perivascular mild mononuclear cellular infiltration in superficial dermis (figure 3). There was focal parakeratosis and mild acanthosis in epidermis. Direct immunofluorescence studies did not reveal any immunoglobulin or complement deposition.

We treated the patient with topical emollient application but it had no effect on his dermatosis.

Discussion

While in 1982, Levy and Goldsmith [7] introduced the term ‘peeling skin syndrome’ for this disease, some other authors used such terms as ‘keratolysis exfoliativa congenita’ [8], ‘familial continual skin peeling’ [9], ‘continual skin peeling syndrome’ [10], ‘deciduous skin’ [11], or ‘idiopathic deciduous skin’ [12] for similar cases.

Rare features of peeling skin syndrome include easily removed hairs, koilonychia, distal onycholysis, chapping, and keratoderma [1]. Our case had distal onycholysis on his fingernails. However, we do not know whether his onycholysis was related with the periodical epidermal shedding or not, because we were able to follow the patient only during one peeling episode. There is usually no systemic abnormality in this syndrome [1, 6]. However, a few reports state sexual dysfunction, anosmia, short stature, primary amenorrhea, and sexual infantilism. Low plasma tryptophan levels, aminoaciduria, and elevated levels of serum IgE, copper, ceruloplasmin, iron and iron binding capacity, and abnormal epidermal retinoid metabolism in some cases have also been reported [1, 6, 13]. Our patient showed no abnormality.

To date, the cause of this disorder has still not been understood completely. Therefore the clinical features of the disease have remained the mainstay of its description. The skin peeling could be either continuous or periodic, and in some cases, seasonal changes have been reported. In most cases of peeling skin syndrome, the separation of the skin is usually generalized. However, in recent years, some patients with localized acral peeling skin syndrome have been reported as well [1, 2, 14]. The palms and soles may be hyperkeratotic but are spared from peeling in most cases with generalized peeling skin syndrome [3]. Our patient had periodic and general skin shedding, and showed palm and sole involvement similar to the involvement Tolat et al.’s patient had [3].

Previous reports proposed a classification including two types (type A or noninflammatory type and type B or inflammatory type) depending on clinical, biological, and pathologic criteria. It has been described that both types have generalized desquamation, sparing palms and soles because acral peeling syndrome was not yet described at the classification time. The peeling begins at birth or at 3 to 6 years of age in type A, and it always begins at birth in type B. Type A includes asymptomatic and noninflammatory exfoliation, and general health usually remains intact. This type may consist of some hyperpigmentation, but no vesiculation or erythema. Type B includes seasonal variation or periodic peeling with inflammatory changes and pruritus. This type shows erythematous plaques with rare vesiculation, and it may be associated with noncutaneous anomalies and abnormal laboratory findings. Histologically, while type A shows ortohyperkeratosis with a normal epidermis, type B shows a psoriasiform pattern [1, 2, 4, 15]. Most of the features of our case such as the presence of periodic peeling with erythema, seasonal variation, and pruritus were like those of type B. However, the onset age of our case was different from that in type B. Desquamation pattern in our patient was also different from that in both types because both types were described as sparing the palms and soles. Probably, generalized peeling skin syndrome has more than two phenotypes. In addition, recently, cases termed as acral peeling syndrome characterized by peeling skin of mainly dorsal and volar surfaces of the hands and feet have been reported. Separation of the stratum corneum from the stratum granulosum is the distinctive feature of peeling skin syndrome which is also the distinctive feature of acral peeling syndrome [1, 2, 14]. Acral skin syndrome seems to be a new variant which differentiates from the type A and B of peeling skin syndrome in that acral peeling syndrome involves only acral regions.

Epidermolysis bullosa superficialis described as a variant of epidermolysis bullosa shows subcorneal cleavage similar to that in peeling skin syndrome. However, presence of an autosomal dominant mode of inheritance, the presence of blistering as a predominant feature of all patients, the absence of spontaneous exfoliation or peeling, and the fact that the onset is always observed before 2 years of age differentiates this disease from peeling skin syndrome [16].

To date, several cases with different clinical features called peeling skin syndrome have been described [1-14, 17, 18]. Since identification of gene defects does not exist, it is difficult to claim that all these cases are the same dermatoses and therefore it is difficult to classify this disorder. Ultrastructural, biochemical, or genetic marker studies might assist us to understand whether these cases are the same dermatosis.

References

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