与银屑病红皮病相关的表皮下大疱性皮肤病中针对BP180和层粘连蛋白γ-1多个表位的自身抗体

Autoantibodies Against Multiple Epitopes in Bp180 and Laminin Gamma-1 in Subepidermal Blistering Skin Disease Associated with Psoriatic Erythroderma.

作者信息

Goto-Hamano Haruna, Ito Keiko, Sakamoto-Kimura Kumiko, Terui Tadashi, Ohyama Bungo, Hashimoto Takashi, Hara Hiroyuki

机构信息

Department of Dermatology, Kawaguchi Municipal Medical Center, 180 Nishiaraijyuku, Kawaguchi, Saitama 333-0833, Japan ; Division of Cutaneous Science, Nihon University School of Medicine, 30-1 Oyaguchi-Kamicho, Itabashi-ku, Tokyo 173-0032, Japan.

Department of Dermatology, Kawaguchi Municipal Medical Center, 180 Nishiaraijyuku, Kawaguchi, Saitama 333-0833, Japan.

出版信息

Indian J Dermatol. 2015 Sep-Oct;60(5):521. doi: 10.4103/0019-5154.164433.

We report a 79-year-old Japanese man who developed subepidermal blistering skin disease after an 8-year history of psoriasis. Histology of a bullous lesion revealed a subepidermal blister with a mixed inflammatory cell infiltrate and fibrin nets. Indirect immunofluorescence using normal human skin sections revealed IgG and IgA autoantibodies in the patient serum, which bound to the epidermal side of 1M NaCl-split skin sections. Immunoblot analysis revealed that both IgA and IgG antibodies reacted with the BP180 NC16a domain and the 120-kDa LAD-1 and that IgG antibodies also reacted with the BP180 C-terminal domain and laminin gamma-1. These findings indicated that autoantibodies to laminin gamma-1 and multiple epitopes in BP180 ectodomain played a role in the pathogenesis of this unique autoimmune subepidermal blistering skin disease associated with psoriasis.

我们报告了一名79岁的日本男性，他在患银屑病8年后出现了表皮下大疱性皮肤病。一个大疱性病变的组织学检查显示为表皮下大疱，伴有混合性炎症细胞浸润和纤维蛋白网。使用正常人皮肤切片进行间接免疫荧光检查发现患者血清中有IgG和IgA自身抗体，它们与1M NaCl分离皮肤切片的表皮侧结合。免疫印迹分析显示，IgA和IgG抗体均与BP180 NC16a结构域以及120-kDa LAD-1发生反应，并且IgG抗体还与BP180 C末端结构域和层粘连蛋白γ-1发生反应。这些发现表明，针对层粘连蛋白γ-1和BP180胞外结构域中多个表位的自身抗体在这种与银屑病相关的独特自身免疫性表皮下大疱性皮肤病的发病机制中起作用。