Pemphigus.

Abstract

Pemphigus is an autoimmune intraepithelial blistering disease of the skin and mucous membranes that is characterized in part by the presence of circulating IgG autoantibodies. These autoantibodies, which have been shown to be pathogenic, bind to complexes on the keratinocyte cell surface and have been utilized to establish that these complexes contain components found in cell adhesion junctions. Although the stimulus for autoantibody production is unknown, proposed mechanisms to explain the disease pathophysiology include proteinase activation, complement activation, and direct interference of cell adhesion junction assembly by autoantibodies. Although pemphigus vulgaris was commonly fatal prior to the availability of glucocorticosteroids, the prognosis has improved dramatically since their introduction. Immunosuppressive agents have also been successfully employed to treat patients with more severe disease.