title: Subepidermal blistering disease with autoantibodies against a novel dermal 200-kDa antigen.
abstract: A number of autoimmune subepidermal blistering diseases are characterized by the distinct autoantigens of the cutaneous basement membrane zone. Recently, a few cases with autoantibodies against a novel 200-kDa dermal protein have been reported. We collected nine cases of subepidermal blistering disease with IgG antibodies against this 200-kDa antigen. In this report, we describe the clinical and immunological appearances in these cases. Five cases showed bullous pemphigoid-like features, one case resembled dermatitis herpetiformis, and another case showed mixed features of bullous pemphigoid and linear IgA bullous dermatosis. It was interesting to note that psoriasis coexisted in four cases. By indirect immunofluorescence on 1 M NaCl split skin, IgG antibodies from all sera reacted with the dermal side of the split. By immunoblot analysis, IgG antibodies recognized a 200-kDa protein of dermal extract. IgG affinity-purified antibodies on the 200-kDa immunoblot membrane stained the dermal side of 1 M NaCl split skin. Various examinations suggested that the 200-kDa antigen is not identical to any chains of laminins-1, -5 or -6. This autoimmune subepidermal blistering disease against the dermal 200-kDa protein may form a new distinct entity, which often associates with psoriasis.
authors.affiliation: Department of Dermatology, Keiyu Hospital, 3-7-3 Minatomirai, Nishi-ku, Yokohama, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Kawahara
authors.forename: D
authors.initials: D
authors.lastname: Zillikens
authors.forename: K B
authors.initials: KB
authors.lastname: Yancey
authors.forename: M P
authors.initials: MP
authors.lastname: Marinkovich
authors.forename: Z
authors.initials: Z
authors.lastname: Nie
authors.forename: T
authors.initials: T
authors.lastname: Hashimoto
authors.forename: T
authors.initials: T
authors.lastname: Nishikawa
mesh_terms: D000328:Adult; D000368:Aged; D000941:Antigens / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000097:blood / Q000276:immunology*; D002478:Cells, Cultured; D003874:Dermatitis Herpetiformis / Q000097:blood / Q000276:immunology; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D015603:Keratinocytes / Q000276:immunology; D008297:Male; D008875:Middle Aged; D008970:Molecular Weight; D010391:Pemphigoid, Bullous / Q000097:blood / Q000276:immunology; D012867:Skin / Q000276:immunology*; D012872:Skin Diseases, Vesiculobullous / Q000097:blood / Q000276:immunology*
chemical_list: D000941:Antigens; D001323:Autoantibodies; D007074:Immunoglobulin G