title: A case of subepidermal blistering disease with autoantibodies to multiple laminin subunits who developed later autoantibodies to alpha-5 chain of type IV collagen associated with membranous glomerulonephropathy.
abstract: We report a 68-year-old Japanese female patient with subepidermal blistering disease with autoantibodies to multiple laminins, who subsequently developed membranous glomerulonephropathy. At skin disease stage, immunofluorescence demonstrated IgG anti-basement membrane zone antibodies reactive with dermal side of NaCl-split skin. Immunoblotting of human dermal extract, purified laminin-332, hemidesmosome-rich fraction and laminin-521 trimer recombinant protein (RP) detected laminin γ-1 and α-3 and γ-2 subunits of laminin-332. Three years after skin lesions disappeared, nephrotic symptoms developed. Antibodies to α-3 chain of type IV collagen (COL4A3) were negative, thus excluding the diagnosis of Goodpasture syndrome. All anti-laminin antibodies disappeared. Additional IB and ELISA studies of RPs of various COL4 chains revealed reactivity with COL4A5, but not with COL4A6 or COL4A3. Although diagnosis of anti-laminin γ-1 (p200) pemphigoid or anti-laminin-332-type mucous membrane pemphigoid could not be made, this case was similar to previous cases with autoantibodies to COL4A5 and/or COL4A6.
authors: Department of Dermatology, Showa University School of Medicine, 1-5-8, Hatanodai, Tokyo 142-8555, Japan.
authors: Hirohiko
authors: H
authors: Sueki
authors: Yoshinori
authors: Y
authors: Sato
authors: Shinpei
authors: S
authors: Ohtoshi
authors: Tokio
authors: T
authors: Nakada
authors: Ashio
authors: A
authors: Yoshimura
authors: Chiharu
authors: C
authors: Tateishi
authors: Dorin-Bogdan
authors: DB
authors: Borza
authors: William
authors: W
authors: Fader
authors: Reza F
authors: RF
authors: Ghohestani
authors: Yoshiaki
authors: Y
authors: Hirako
authors: Hiroshi
authors: H
authors: Koga
authors: Norito
authors: N
authors: Ishii
authors: Atsunari
authors: A
authors: Tsuchisaka
authors: Hua
authors: H
authors: Qian
authors: Xiaoguang
authors: X
authors: Li
authors: Takashi
authors: T
authors: Hashimoto
mesh_terms: D000368:Aged; D001323:Autoantibodies / Q000032:analysis* / Q000097:blood; D001327:Autoimmune Diseases / Q000097:blood / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D001706:Biopsy; D001768:Blister / Q000097:blood / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D024141:Collagen Type IV / Q000276:immunology*; D005260:Female; D005455:Fluorescent Antibody Technique; D015433:Glomerulonephritis, Membranous / Q000097:blood / Q000175:diagnosis / Q000276:immunology*; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007668:Kidney / Q000276:immunology* / Q000648:ultrastructure; D007797:Laminin / Q000276:immunology*; D010951:Plasma Exchange; D011237:Predictive Value of Tests; D021122:Protein Subunits; D012867:Skin / Q000187:drug effects / Q000276:immunology* / Q000473:pathology; D013997:Time Factors
chemical_list: D001323:Autoantibodies; C478159:COL4A5 protein, human; D024141:Collagen Type IV; D005938:Glucocorticoids; D007797:Laminin; D021122:Protein Subunits