Pemphigus herpetiformis: From first description until now - 14/03/14
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Abstract |
Pemphigus herpetiformis is one of the less common forms of pemphigus, first introduced by Jabłońska and colleagues in 1975. This autoimmune bullous disease combines the clinical features of dermatitis herpetiformis and the immunologic characteristics of pemphigus. The target autoantigen is usually desmoglein 1 (or less frequently desmoglein 3), although recently it has become increasingly obvious that patients with pemphigus herpetiformis also demonstrate autoantibodies against desmocollin. Here, we summarize reported cases of pemphigus herpetiformis and describe current knowledge considering epidemiology, clinical manifestations, histologic findings, immunopathology, pathophysiologic concepts, associated diseases, and treatment of this rare disorder.
Le texte complet de cet article est disponible en PDF.Key words : autoantibody, autoantigen, autoimmunity, bullous disease, desmoglein, pemphigus
Abbreviations used : Dsc, Dsg, PF, PH, PV
Plan
Funding sources: None. |
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Conflicts of interest: None declared. |
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Reprints not available from the authors. |
Vol 70 - N° 4
P. 780-787 - avril 2014 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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