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

作者：Sueki Hirohiko; Sato Yoshinori; Ohtoshi Shinpei; Nakada Tokio; Yoshimura Ashio; Tateishi Chiharu; Borza Dorin Bogdan; Fader William; Ghorestani Reza F; Hirako Yoshiaki; Koga Hiroshi; Ishii Norito; Tsuchisaka Atsunari; Qian Hua; Li Xiaoguang; Hashimoto Takashi^*

来源：ACTA DERMATO-VENEREOLOGICA, 2015, 95(7): 826-829.

DOI：10.2340/00015555-2059

• autoimmune hemidesmosome
• laminin
• membranous glomerulonephropathy
• type IV collagen
• subepidermal bullous disease

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 gamma-1 and alpha-3 and gamma-2 subunits of laminin-332. Three years after skin lesions disappeared, nephrotic symptoms developed. Antibodies to alpha-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 gamma-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.

• 出版日期2015