Peeling Skin Syndrome: Case Report

Abstract

Peeling skin syndrome (PSS) is a rare genodermatosis of autosomal recessive inheritance caused by mutations in CDSN, TGM5 or CHST8, depending on the type of disease. It is characterized by localized or generalized superficial peeling of the skin, with or without erythema and itching. Concerning to the generalized form, Heiko Traupe proposed distinction between noninflammatory (type A) and associated with congenital erythroderma (type B). Mevorah described the type C, with features similar to type B. Noninflammatory type is characterized for continuous spontaneous clearance areas of stratum corneum, which begins at birth or during childhood. We describe a 19 year’s old male patient, with skin peeling of the neck, trunk and upper limbs since childhood and a similar dermatoses in the family.