A Case of Epidermolysis Bullosa Dystrophica / 대한피부과학회지

ABSTRACT

Epidermolysis bullosa dystrophica is a rare, chronic non-infIammatory mechanobullous disease of hereditsry trait, which easily produces bulla by minor injury or spontaneously. Bullae usually developed first at birth or in infancy, involving especially hands, feet, knees, elbows and other pressure sites, which heal with atrophic and hypertrophic scars, loss of hair and nails, digital fusion, flexural contractures of the knee, elbow and wrist joints. A 5-year-old boy has had various sized recurrent buIlae on hands, feet, kneces and elbows, healed with atrophy of skin, miliums, deformities of toe and finger nails. Diagnosis was confirmed by clinical features and electronmicroscopic findings. Literature were reviewed briefly.