title: The pathogenic role of autoantibodies in pemphigus vulgaris.
abstract: Pemphigus vulgaris (PV) is a severe autoimmune bullous disease involving both the skin and mucosal areas, and characterized by intraepithelial flaccid blisters and erosions. The pathogenesis of this disease is not yet completely understood, but novel insights into desmoglein biology and autoantibody pathogenesis have recently been published. Acantholysis in PV seems to result from a collective action of autoantibodies against various keratinocyte self antigens, of which desmogleins 1 and 3 are the most important. Additional antigens including desmocollins and nondesmosome components, such as the mitochondrion, might take part in disease activation. Recently, apoptosis was reported as a possible underlying mechanism of acantholysis. Furthermore, apoptolysis is believed to be the link between suprabasal acantholytic and cell-death pathways. We review the possible hypotheses of the pathogenesis of PV: the desmoglein compensation theory, the antibody-induced apoptosis theory, the basal-cell shrinkage hypothesis and the newly published apoptolysis theory.
authors: Department of Dermatology, Ruijin Hospital, School of Medicine, Jiao Tong University, Shanghai, China.
authors: M
authors: M
authors: Pan
authors: X
authors: X
authors: Liu
authors: J
authors: J
authors: Zheng
mesh_terms: D000051:Acantholysis / Q000276:immunology; D017209:Apoptosis / Q000276:immunology; D001323:Autoantibodies / Q000276:immunology*; D051187:Desmocollins / Q000276:immunology; D051182:Desmogleins / Q000276:immunology; D006801:Humans; D015603:Keratinocytes / Q000276:immunology; D008928:Mitochondria / Q000276:immunology; D010392:Pemphigus / Q000276:immunology*
chemical_list: D001323:Autoantibodies; D051187:Desmocollins; D051182:Desmogleins