Karatolysis Exafoliative Congenitum

Abstract

A middle aged male had keratolysis exfoliative congenitum (continual skin peeling), since his infancy. None of his family members was affected by a similar skin disease. Mild rubbing of the skin with fingers caused rapid peeling of the horny layer. Oral vitamin A,50,000unitsdailyfortwomonthsand topical liquid paraffin were ineffective in controlling the desquamation.