title: Update on the pathogenesis of bullous pemphigoid: an autoantibody-mediated blistering disease targeting collagen XVII.
abstract: Bullous pemphigoid (BP) is a common autoimmune blistering skin disorder that tends to affect the elderly. Autoantibodies (autoAbs) from BP patients react with two hemidesmosomal components: transmembrane collagen XVII (BP180 or BPAG2) and plakin family protein BP230 (BPAG1). Of these, collagen XVII (COL17) is thought to be a major autoantigen. The binding of autoAbs to COL17 following the activation of complements and inflammatory pathways eventually leads to the degradation of COL17, and this has been regarded as the main pathogenesis of BP. However, recent investigations have suggested other pathways, including a complement-independent pathway and a pathway involving IgE-autoAbs. BP-autoAbs can directly deplete COL17, leading to fragility of the dermal-epidermal junction. In addition, IgE-autoAbs to COL17 may be involved in the formation of itchy urticarial erythema associated with eosinophilic infiltration. This article summarizes the update on pathogenesis of BP, with a special focus on blister formation by autoAbs to COL17.
authors.affiliation: Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan. Electronic address: nishie@med.hokudai.ac.jp.
authors.forename: Wataru
authors.initials: W
authors.lastname: Nishie
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D001323:Autoantibodies / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology* / Q000378:metabolism; D003165:Complement System Proteins / Q000276:immunology; D006801:Humans; D007073:Immunoglobulin E / Q000276:immunology; D007249:Inflammation / Q000209:etiology; D008969:Molecular Sequence Data; D024041:Non-Fibrillar Collagens / Q000276:immunology* / Q000378:metabolism; D010391:Pemphigoid, Bullous / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D000097805:Collagen Type XVII
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D003165:Complement System Proteins; D007073:Immunoglobulin E; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII
keywords: Autoantibody; Complement activation; IgE; Model mice; Protease