Acral peeling skin syndrome

Peeling skin syndrome is a rare autosomal recessive disease characterized b y widespread painless peeling of the skin in superficial sheets. We describ e a 34-year-old man with a lifelong history of spontaneous asymptomatic pee ling skin limited to the acral surfaces. This patient probably represents a localized variant of peeling skin syndrome, which has previously been desc ribed as a generalized condition. Light and electron microscopic studies of biopsy specimens taken before and after immersion in water were performed. It was concluded that this patient has abnormal keratohyalin granules and inadequate aggregation of keratin filaments that caused the separation of t he epidermis in the stratum corneum through the clear zone. Alternatively, unknown keratin species expressed in the clear zone may also cause the abno rmality.