title: Bullous pemphigoid: role of complement and mechanisms for blister formation within the lamina lucida.
abstract: Bullous pemphigoid (BP), an autoimmune subepidermal blistering skin disease, demonstrates tense blisters with or without widespread erythema, blistering along the lamina lucida, immunoglobulin G and/or complement deposits at the basement membrane zone, and the presence of circulating autoantibodies against hemidesmosomal molecules. These autoantibodies usually react against 180-kDa and/or 230-kDa proteins, designated as BP180 and BP230, respectively. The precise blistering mechanisms after autoantibodies bind to antigens are not fully understood. Immune complexes are thought to initially activate the complement cascade, which may induce activation of proteases and/or cytokines and cause dermal-epidermal separation. However, why does separation run specifically within the lamina lucida in a space as narrow as 500 nm wide? This review mainly focuses on the possible mechanisms of BP-specific blistering and how separation occurs along the lamina lucida, based on existing evidence.
authors: Department of Dermatology, University of Lübeck, Lübeck, Germany. hiroaki.iwata@uk-sh.de
authors: Hiroaki
authors: H
authors: Iwata
authors: Yasuo
authors: Y
authors: Kitajima
mesh_terms: D000818:Animals; D001323:Autoantibodies / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology; D001485:Basement Membrane / Q000276:immunology; D001768:Blister / Q000276:immunology*; D002462:Cell Membrane / Q000378:metabolism; D003167:Complement Activation; D003165:Complement System Proteins / Q000276:immunology*; D016207:Cytokines / Q000378:metabolism; D004817:Epidermis / Q000276:immunology; D022002:Hemidesmosomes / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D007249:Inflammation; D051379:Mice; D024041:Non-Fibrillar Collagens / Q000276:immunology; D010391:Pemphigoid, Bullous / Q000175:diagnosis* / Q000276:immunology* / Q000503:physiopathology; D010447:Peptide Hydrolases / Q000378:metabolism; D000097805:Collagen Type XVII
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D003165:Complement System Proteins; D016207:Cytokines; D007074:Immunoglobulin G; D024041:Non-Fibrillar Collagens; D010447:Peptide Hydrolases; D000097805:Collagen Type XVII