Peeling skin syndrome

Peeling skin syndrome is a rare autosomal recessive disease characterized histologically by separation of the stratum corneum above the stratum granulosum. The cardinal clinical features are generalized, inflammatory, spontaneous, continual peeling of the skin and pruritus. Other historic and clinical findings include a positive family history, parentalconsanguini ty, short stature, primaryamenorrhea, sexual infantilism, anosmia, easily removed hairs, palmoplantar keratoderma with chapping, palmoplantar erythema, distal onycholysis,and koilonychia. 1-5 Aminoaciduria, low plasma tryptophan levels,and elevated levels of serum iron, copper, and 19E have also been observed.2-5 We describe a patient with peeling skin syndrome and provide a review of the literature.