PubMed:19084695 JSONTXT 9 Projects

[Juvenile pemphigus vulgaris]. Pemphigus vulgaire de l'enfant. BACKGROUND: Pemphigus vulgaris is a rare autoimmune blistering disease seen only, rarely, in children. CASE-REPORT: Two young girls, aged four and 15 years respectively, presented with oral and/or cutaneous blisters (case 2). The diagnosis of pemphigus vulgaris was confirmed by histology (suprabasal acantholysis) and immunopathological analysis (direct and indirect immunofluorescence, antidesmoglein-3 plus or minus antidesmoglein-1 antibodies by ELISA). In case 2, caused by corticosteroid dependence and after the failure of intravenous immunoglobulin, the patient was successfully treated with rituximab. DISCUSSION: Juvenile pemphigus vulgaris is rare and its occasionally clinically atypical presentation can result in delayed diagnosis and management. Firstline therapy generally comprises systemic corticosteroids. Some cases of recalcitrant pemphigus vulgaris in adults with a refractory course, despite adequate systemic corticosteroids, have been successfully treated with rituximab, as in our case 2. To our knowledge, only two other cases of juvenile pemphigus vulgaris treated with rituximab have been reported.