Sueki Hirohiko, Sato Yoshinori, Ohtoshi Shinpei, Nakada Tokio, Yoshimura Ashio, Tateishi Chiharu, Borza Dorin-Bogdan, Fader William, Ghohestani Reza F, Hirako Yoshiaki, Koga Hiroshi, Ishii Norito, Tsuchisaka Atsunari, Qian Hua, Li Xiaoguang, Hashimoto Takashi
Department of Dermatology, Showa University School of Medicine, 1-5-8, Hatanodai, Tokyo 142-8555, Japan.
Acta Derm Venereol. 2015 Sep;95(7):826-9. doi: 10.2340/00015555-2059.
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.
我们报告了一名68岁的日本女性患者，患有伴有多种层粘连蛋白自身抗体的表皮下大疱性疾病，随后发展为膜性肾小球肾炎。在皮肤病阶段，免疫荧光显示IgG抗基底膜带抗体与经氯化钠分离的皮肤的真皮侧发生反应。对人真皮提取物、纯化的层粘连蛋白-332、富含半桥粒的组分和层粘连蛋白-521三聚体重组蛋白（RP）进行免疫印迹检测到层粘连蛋白γ-1以及层粘连蛋白-332的α-3和γ-2亚基。皮肤病变消失三年后，出现肾病症状。IV型胶原α-3链（COL4A3）抗体为阴性，因此排除了Goodpasture综合征的诊断。所有抗层粘连蛋白抗体均消失。对各种COL4链的RP进行的额外免疫印迹和酶联免疫吸附试验研究显示与COL4A5有反应，但与COL4A6或COL4A3无反应。虽然无法诊断抗层粘连蛋白γ-1（p200）类天疱疮或抗层粘连蛋白-332型黏膜类天疱疮，但该病例与先前有针对COL4A5和/或COL4A6自身抗体的病例相似。
