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      Mechanisms of Disease: Pemphigus and Bullous Pemphigoid

      research-article
      1 , 2 , 1
      Annual review of pathology
      autoimmune, autoantibody, blistering, skin

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          Abstract

          Pemphigus and bullous pemphigoid are autoantibody-mediated blistering skin diseases. In pemphigus, keratinocytes in epidermis and mucous membranes lose cell-cell adhesion, and in pemphigoid, the basal keratinocytes lose adhesion to the basement membrane. Pemphigus lesions are mediated directly by the autoantibodies, whereas the autoantibodies in pemphigoid fix complement and mediate inflammation. In both diseases, the autoantigens have been cloned and characterized; pemphigus antigens are desmogleins (cell adhesion molecules in desmosomes), and pemphigoid antigens are found in hemidesmosomes (which mediate adhesion to the basement membrane). This knowledge has enabled diagnostic testing for these diseases by enzyme-linked immunosorbent assays and dissection of various pathophysiological mechanisms, including direct inhibition of cell adhesion, antibody-induced internalization of antigen, and cell signaling. Understanding these mechanisms of disease has led to rational targeted therapeutic strategies.

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          Author and article information

          Journal
          101275111
          33888
          Annu Rev Pathol
          Annu Rev Pathol
          Annual review of pathology
          1553-4006
          1553-4014
          23 July 2017
          22 February 2016
          23 May 2016
          17 August 2017
          : 11
          : 175-197
          Affiliations
          [1 ]Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania 19104
          [2 ]Department of Dermatology, University of Luebeck, D-23562 Luebeck, Germany
          Article
          PMC5560122 PMC5560122 5560122 nihpa894206
          10.1146/annurev-pathol-012615-044313
          5560122
          26907530
          00335cb4-cf8d-4bd3-a199-33e1cc14e8b0
          History
          Categories
          Article

          autoimmune,skin,blistering,autoantibody
          autoimmune, skin, blistering, autoantibody

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