Epidermolysis bullosa: a review

Epidermolysis bullosa is a group of inherited disorders characterized by blistering of the skin as a result of minor trauma. The disease can be divided into three anatomical categories: Epidermolytic, where blister cleavage occurs within the epidermis; Junctional, which has blister cleavage within the lamina lucida; and Dermolytic, where blister cleavage occurs below the basal lamina in the upper papillary dermis. Each of these three categories can be divided into several distinct entities based on clinical and histologic criteria. Basic biochemical studies have increased our understanding of several of these diseases, most notably recessive dystrophic epidermolysis bullosa. Although therapy for patients with EB is largely supportive, increased knowledge of the biochemistry of these disorders is making direct therapeutic interventions possible.