title: From anti-p200 pemphigoid to anti-laminin gamma1 pemphigoid.
abstract: Anti-laminin gamma1 pemphigoid is an autoimmune subepidermal bullous disease first described in 1996, and has been distinct from previously known subepidermal blistering diseases, such as bullous pemphigoid and epidermolysis bullosa acquisita. Circulating autoantibodies of the patients do not react to any known autoantigen of the skin, but react to a 200-kDa molecule (p200) from dermal extracts. The identity of p200 was unmasked as laminin gamma1, an extracellular matrix glycoprotein composing several forms of laminin heterotrimers. We renamed this disease from the previously used anti-p200 pemphigoid to anti-laminin gamma1 pemphigoid, a new entity of an autoimmune bullous disease. In this decade, we have experienced over 70 cases of this disease. Although the number of the cases of anti-laminin gamma1 pemphigoid is half as many as the number of definitely diagnosed cases of epidermolysis bullosa acquisita in the same duration, a considerable number of the cases could be clinically misdiagnosed as epidermolysis bullosa acquisita. Unveiling the pathogenicity and development of a useful diagnostic method is necessary for appropriate management of this new disease.
authors.affiliation: Department of Dermatology, Kurume University School of Medicine, Kurume, Fukuoka, Japan.
authors.forename: Teruki
authors.initials: T
authors.lastname: Dainichi
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Koga
authors.forename: Takako
authors.initials: T
authors.lastname: Tsuji
authors.forename: Norito
authors.initials: N
authors.lastname: Ishii
authors.forename: Bungo
authors.initials: B
authors.lastname: Ohyama
authors.forename: Akihiro
authors.initials: A
authors.lastname: Ueda
authors.forename: Yohei
authors.initials: Y
authors.lastname: Natsuaki
authors.forename: Tadashi
authors.initials: T
authors.lastname: Karashima
authors.forename: Takekuni
authors.initials: T
authors.lastname: Nakama
authors.forename: Shinichiro
authors.initials: S
authors.lastname: Yasumoto
authors.forename: Detlef
authors.initials: D
authors.lastname: Zillikens
authors.forename: Takashi
authors.initials: T
authors.lastname: Hashimoto
mesh_terms: D000368:Aged; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D001324:Autoantigens / Q000276:immunology / Q000302:isolation & purification; D001485:Basement Membrane / Q000276:immunology; D001768:Blister / Q000276:immunology / Q000473:pathology; D006801:Humans; D007797:Laminin / Q000276:immunology*; D008875:Middle Aged; D020556:Neutrophil Infiltration; D010391:Pemphigoid, Bullous / Q000276:immunology* / Q000473:pathology
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D007797:Laminin; C089141:laminin gamma 1