Epidermolysis bullosa hereditaria letalis: PATHOLOGY, NATURAL HISTORY AND THERAPY

LAWRENCE SCHACHNER, GERALD S. LAZARUS, HERBERT DEMBITZER

Research output: Contribution to journalArticlepeer-review

45 Scopus citations

Abstract

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 patient 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.

Original languageEnglish (US)
Pages (from-to)51-58
Number of pages8
JournalBritish Journal of Dermatology
Volume96
Issue number1
DOIs
StatePublished - Jan 1977
Externally publishedYes

ASJC Scopus subject areas

  • Dermatology

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