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Updated: May 29, 2026

Granulocyte-dependent Autoantibody-induced Skin Blistering
Published on: October 12, 2012
[Bullous pemphigoid, primary biliary cirrhosis and vitiligo: a multiple autoimmune syndrome?]
Benjamin Marcet1, Vincent Sibaud, Marc Géniaux
1Service de Dermatologie, Hôpital Saint-André, CHU de -Bordeaux, 33075 Bordeaux Cedex, France.
Abstract:
Bullous pemphigoid is the most frequent autoimmune blistering skin disease. There have been few reports of an association with primary biliary cirrhosis and vitiligo. We report the simultaneous occurrence of bullous pemphigoid and primary biliary cirrhosis in an 86-year-old patient who also suffered from vitiligo. Multiple autoimmune syndrome, proposed by Humbert and Dupond, can be divided into three groups based on preferential associations of autoimmune disorders. The association of bullous pemphigoid, cirrhosis biliary primary and vitiligo has been reported three times in the literature. This association is probably not fortuitous and suggests a pathogenic relationship. This association is not typical of the multiple autoimmune syndrome as defined by Humbert and Dupond but the collection of such observations may contribute to revise the classification of autoimmune disease and provide a better understanding of the pathophysiological mechanisms of autoimmunity.
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