Generalized peeling skin syndrome: Case report and review of the literature.
Peeling skin syndrome (PSS) is a rare form of ichthyosis with a probable autosomal recessive inheritance that exhibits superficial, painless, continual, or seasonal cutaneous exfoliation. The syndrome generally appears at birth or in infancy. We report a case of generalized PSS and provide a literature review. A 34-year-old woman reported a lifelong history of generalized and painless peeling of the skin that worsened in summer. Her parents were third degree cousins. Her twin sister and her two cousins presented with the same condition. Physical examination showed widespread superficial sheets of variable size that could be easily removed without bleeding or pain. No underlying erythema was noted. Otherwise, the patient was in good health. Histological findings showed an epidermal cleavage within the stratum corneum. The generalized form of PSS is classified into 3 types, A, B, and C, according to the classification system of Traupe and Mevorah. We have tried to classify the cases of generalized PSS already reported in the literature into one of these three types. Thirteen reported cases probably presented PSS-type A. Sixteen patients are best described as PSS-type B. Two patients exhibit PSS-type C. Fifteen reported patients had an acral form of peeling skin syndrome. The classification of the eleven remaining patients was difficult to determine. Our patient presented clinical and histological features of generalized PSS-type A.
Kharfi
Monia
M
Department of Dermatology, Charles Nicolle Hospital, Tunis, Tunisia.
Khaled
Aida
A
Ammar
Donia
D
Ezzine
Nadia
N
El Fekih
Nadia
N
Fazaa
Becima
B
Jaafoura
Hiabib
H
Kamoun
Mohamed Ridha
MR
D000328:Adult; D003241:Consanguinity; D005260:Female; D006801:Humans; D007057:Ichthyosis / Q000145:classification* / Q000235:genetics / Q000473:pathology*; D013577:Syndrome; D011503:Transglutaminases / Q000235:genetics
C418703:transglutaminase 5; D011503:Transglutaminases