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.

Autor: Hirohiko SUEKI, Yoshinori SATO, Shinpei OHTOSHI, Tokio NAKADA, Ashio YOSHIMURA, Chiharu TATEISHI, BORZA, Dorin-Bogdan, FADER, William, GHOHESTANI, Reza F., Yoshiaki HIRAKO, Hiroshi KOGA, Norito ISHII, Atsunari TSUCHISAKA, Hua QIAN, Xiaoguang LI, Takashi HASHIMOTO
Předmět:
Zdroj: Acta Dermato-Venereologica; Sep2015, Vol. 95 Issue 7, p826-829, 4p
Abstrakt: 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. [ABSTRACT FROM AUTHOR]
Databáze: Complementary Index