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Article
May 1988

Pathogenesis, Clinical Features, and Management of the Non-Dermatological Complications of Epidermolysis Bullosa

Author Affiliations

Department of Pathology University of Vermont Medical Alumni Building Burlington, VT 05405

Arch Dermatol. 1988;124(5):705. doi:10.1001/archderm.1988.01670050049019

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Abstract

Epidermolysis bullosa (EB) is not one but a family of disorders that share the common feature of reduced resistance to shear and frictional injury. The basic lesions of the different forms occur at various anatomic sites in the epidermal and subepidermal tissues. The multiple types of EB have been classified on the basis of genetic modes of inheritance, the anatomic location of the lesions and their distribution, and the morbidity associated with the illness.

Although EB is considered a skin disease, lesions of internal organs also develop, resulting in a variety of medical complications. The literature tends to emphasize the more obvious lesions of the skin, sometimes to the exclusion of the less apparent, but troublesome and sometimes devastating, changes in other body systems. Recognizing this, in September 1986, the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) funded a workshop (Public Health Service Grant R13-36465-01) with the

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