A Case of Nonscarring Subepidermal Blistering Disease Associated with Autoantibodies Reactive with Both Type VII Collagen and Laminin 5
A Case of Nonscarring Subepidermal Blistering Disease Associated with Autoantibodies Reactive with Both Type VII Collagen and Laminin 5
复制标题
与 VII 型胶原蛋白和层粘连蛋白 5 均有反应的自身抗体相关的非疤痕性表皮下水疱病一例
作者：
N. Umemoto;T. Demitsu;S. Toda;Masumi Ohsawa;T. Noguchi;M. Kakurai;Tomoko Yamada;Masayuki Suzuki;H. Nakagawa;A. Komai;T. Hashimoto
一名 35 岁的日本女性，面部、颈部和上背部出现复发性瘙痒性水疱性病变，口腔和生殖器出现糜烂性病变。全身给予皮质类固醇和硫唑嘌呤后，皮肤和粘膜病变愈合，不留疤痕。直接免疫荧光显示 IgG、IgA 和 C3 在皮肤基底膜区域呈线性沉积。 1 M NaCl 分割人体皮肤切片的间接免疫荧光显示，患者的 IgG 抗体与分割的真皮侧发生反应，而 IgA 抗体与表皮侧反应较弱。通过免疫印迹分析，患者的血清与 VII 型胶原蛋白的 NC1 结构域以及层粘连蛋白 5 的 α3 和 β3 亚基发生反应。我们认为该病例是一种非疤痕性表皮下水疱性疾病，具有针对 VII 型胶原蛋白和层粘连蛋白 5 的两个不同亚基的自身抗体。以前从未报道过此类病例。
A 35-year-old Japanese woman had recurrent, pruritic, vesicular lesions on the face, neck and upper back as well as erosive lesions of the oral cavity and genitalia. The skin and mucosal lesions healed without scarring upon the systemic administration of corticosteroid and azathioprine. Direct immunofluorescence revealed linear deposits of IgG, IgA and C3 at the cutaneous basement membrane zone. Indirect immunofluorescence on 1 M NaCl-split human skin sections demonstrated that the patient’s IgG antibodies reacted with the dermal side of the split, while IgA antibodies weakly reacted with the epidermal side. By immunoblot analyses, the patient’s serum reacted with the NC1 domain of type VII collagen as well as both the α3- and β3-subunits of laminin 5. We regarded our case as a nonscarring subepidermal blistering disease with autoantibodies to both type VII collagen and two different subunits of laminin 5. Such a case has not been previously reported.