The inverse form of recessive dystrophic epidermolysis bullosa is a rare genodermatosis characterized by a smouldering course of integumental blistering with improvement of lesions in adulthood, preferential localizations of lesions in flexural areas, severe oral and esophageal mucosal involvement and nail dystrophy. We describe a 41-year-old patient showing all the typical features of this form of epidermolysis bullosa. Ultrastructural findings in specimens obtained from perilesional and healthy skin were similar to those usually observed in the Hallopeau-Siemens form of epidermolysis bullosa. The patient has been treated with phenytoin for a period of 9 months with considerable improvement of the skin manifestations.
|Titolo:||Dystrophic epidermolysis bullosa inversa : a case report|
|Parole Chiave:||Skin; Humans; Adult; Microscopy, Electron; Epidermolysis Bullosa Dystrophica; Male; Blister; Microbial Collagenase|
|Settore Scientifico Disciplinare:||Settore MED/35 - Malattie Cutanee e Veneree|
|Data di pubblicazione:||1990|
|Digital Object Identifier (DOI):||10.1159/000247905|
|Appare nelle tipologie:||01 - Articolo su periodico|