[Bullous pemphigoid, primary biliary cirrhosis and vitiligo: a multiple autoimmune syndrome?]

Ann Med Interne (Paris). 2002 Sep;153(5):349-50.
[Article in French]

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.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Aged
  • Aged, 80 and over
  • Autoimmune Diseases*
  • Azathioprine / administration & dosage
  • Azathioprine / therapeutic use
  • Dermatologic Agents / administration & dosage
  • Dermatologic Agents / therapeutic use
  • Female
  • Humans
  • Immunosuppressive Agents / administration & dosage
  • Immunosuppressive Agents / therapeutic use
  • Liver Cirrhosis, Biliary / complications*
  • Liver Cirrhosis, Biliary / diagnosis
  • Pemphigoid, Bullous / complications*
  • Pemphigoid, Bullous / diagnosis
  • Pemphigoid, Bullous / drug therapy
  • Pemphigoid, Bullous / immunology
  • Syndrome
  • Treatment Outcome
  • Vitiligo / complications*
  • Vitiligo / diagnosis
  • Vitiligo / drug therapy
  • Vitiligo / immunology

Substances

  • Adrenal Cortex Hormones
  • Dermatologic Agents
  • Immunosuppressive Agents
  • Azathioprine