Epidermolysis bullosa hereditaria letalis.
- 1 January 1977
- journal article
- research article
- Published by Oxford University Press (OUP) in British Journal of Dermatology
- Vol. 96 (1), 51-58
- https://doi.org/10.1111/j.1365-2133.1977.tb05185.x
Abstract
A patient [an infant] with epidermolysis bullosa hereditaria letalis had the characteristic EM lesions not only in the skin, but also in the gastrointestinal, genito-urinary and respiratory tracts. Administration of dexamethasone to this patient resulted in a significant decrease in the blistering. Although the patient died, long term survival in this disease is not rare. Staining tissue specimens from patients with epidermolysis bullosa with fluorescein-labeled bullous pemphigoid antibody is a reliable method for differentiating between junctional and dystrophic disease.This publication has 7 references indexed in Scilit:
- Lysosomes And The SkinJournal of Investigative Dermatology, 1975
- Epidermolysis bullosa hereditaria letalis. Clinical and histological manifestations and course of the diseaseArchives of Dermatology, 1974
- Collagenase and Connective Tissue Metabolism in Epidermolysis BullosaJournal of Investigative Dermatology, 1972
- The Oral and Pharyngeal Manifestations of Skin DiseaseProceedings of the Royal Society of Medicine, 1963
- EPIDERMOLYSIS BULLOSA LETALISPediatrics, 1960
- Epidermolysis Bullosa Hereditaria `Letalis': Report of a Case Surviving for Two-and-a-half YearsArchives of Disease in Childhood, 1957
- Kongenitaler, nicht syphilitischer Pemphigus. Eine Ubersicht nebst Beschreibung einer neuen Krankheitsform (Epidermolysis bullosa hereditaria letalis)Acta Paediatrica, 1935