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March 1974

Epidermolysis Bullosa Hereditaria LetalisClinical and Histological Manifestations and Course of the Disease

Author Affiliations


From the Department of Dermatology, Rush-Presbyterian-St. Luke's Medical Center (Dr. Pearson); the Section of Dermatology and Department of Pathology, University of Chicago (Dr. Strauss); and the Department of Dermatology, University of Illinois (Dr. Potter), Chicago.

Arch Dermatol. 1974;109(3):349-355. doi:10.1001/archderm.1974.01630030009001

Five cases of epidermolysis bullosa hereditaria letalis are presented as a basis for discussion of the clinical features and course of the disease. The distribution and morphologic characteristics of the lesions suggest the diagnosis, but histologic findings, and particularly the ultrastructural findings, are crucial to early diagnosis. Blisters form between the plasma membrane of the basal cell and the basement membrane, but the diagnosis can be made from examination of the epidermis alone. The microscopical findings suggest several possibilities concerning the nature of the basic defect; the most plausible is that junction is under attack by lytic enzymes. Most patients live only a few weeks or months, but patients who do survive retain distinct clinical and microscopical features that allow easy differentiation from other mechanobullous diseases.