Epidermolysis bullosa hereditaria letalis: report of a case and probable ultrastructural defects

A case of epidermolysis bullosa hereditaria letalis (Herlitz) is described. In accordance with findings in other cases of Herlitz disease blister formation occurs in form of junctional separation. Electron microscopic study revealed scarcity and abnormal structure of hemidesmosomes, which may play an important role in the pathomorphogenesis of junctional blistering.