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Epidermolysis Bullosa: A Review
Author(s) -
Cooper Thomas W.,
Bauer Eugene A.
Publication year - 1984
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.1984.tb01113.x
Subject(s) - medicine , epidermolysis bullosa , dermatology , junctional epidermolysis bullosa (veterinary medicine) , pathology , dermis , basal lamina , papillary dermis , biology , extracellular matrix , ultrastructure , laminin , microbiology and biotechnology
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; functional, 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.