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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