Epidermolysis Bullosa Hereditaria Letalis

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Abstract
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.

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