Bullous pemphigoid and related subepidermal autoimmune blistering diseases

E. Olasz, K. Yancey

Research output: Contribution to journalArticle

39 Scopus citations

Abstract

The pemphigoid group of autoimmune blistering diseases includes distinct entities (bullous pemphigoid, mucous membrane pemphigoid, pemphigoid gestationis, linear IgA dermatosis and lichen planus pemphigoides) that are characterized by relatively consistent clinical, histologic and immunopathologic findings. Patients with these disorders have antibasement membrane autoantibodies that often display pathogenic (blister-forming) activity following passive transfer to experimental animals. Interestingly, such autoantibodies target important structural proteins that promote adhesion of epidermis to epidermal basement membrane in human skin. Autoimmune blistering diseases are characterized by substantial morbidity (for example pruritus, pain, disfigurement) and in some instances mortality. Treatment with systemic immunosuppressives has reduced morbidity and mortality in patients with these diseases.

Original languageEnglish (US)
Pages (from-to)141-166
Number of pages26
JournalCurrent Directions in Autoimmunity
Volume10
DOIs
Publication statusPublished - 2008

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ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy
  • Biochemistry, Genetics and Molecular Biology(all)

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