Epidermolysis bullosa hereditaria letalis: pathology, natural history and therapy

A patient with epidermolysis bullosa hereditaria letalis had the characteristic electron microscopic lesions not only in the skin, but also in the gastro-intestinal, genito-urinary and respiratory tracts. Administration of dexamethasone to this patients resulted in a significant decrease in the blistering. Although the patient died, long term survival in this disease is not rare. We report that staining tissue specimens from patients with epidermolysis bullosa with fluorescein-labelled bullous pemphigoid antibody is a reliable method for differentiating between junctional and dystrophic disease.