Dystrophic Epidermolysis Bullosa in Two Sisters

Ahn, Byung Jun; Jang, Hyo Chan; Kim, Sang Won; Han, Chi Dong

Abstract

Dystrophic epidermolysis bullosa is a rare, chronic non-inflammatory bullous disease, which easily forms bullae by minor mechanical trauma or spontaneously, is inherited either in an autosomal dominant or autosomal recessive fashion. We report herein two cases which presented with bullae, erosions and ulcers on extremities, buttock, chest, abdomen and face and loss of all nail since birth in two sisters. Bulla occured bencath the basal lamina histopathologically, anchoring fibrils were almost absent on electron miaoscopy in both cases. The two sisters represented dystrophic epidermolysis bullosa considering the absence of family history inheritcd in an autosomal dominant fashion and the clinical, histological and electronmicroscopic findings.