Mechanisms of Disease: Pemphigus and Bullous Pemphigoid

Christoph M. Hammers, John R. Stanley

    90 Citations (Scopus)


    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.

    Original languageEnglish
    JournalAnnual Review of Pathology: Mechanisms of Disease
    Pages (from-to)175-197
    Number of pages23
    Publication statusPublished - 23.05.2016

    Research Areas and Centers

    • Academic Focus: Center for Infection and Inflammation Research (ZIEL)


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