Epidermolysis bullosa hereditaria letalis: pathology, natural history and therapy.

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