Anti-p200 pemphigoid is an autoimmune subepidermal blistering disease characterized by autoantibodies to an unknown 200-kDa acidic noncollagenous glycoprotein of the lower lamina lucida, whereas antilaminin 5 mucous membrane pemphigoid is characterized by autoantibodies to a major basement membrane extracellular matrix, laminin 5. We report a 64-year-old Japanese woman with a subepidermal blistering disease associated with lymph node metastasis of ovarian clear cell carcinoma 10 years after its surgical treatment. Clinical features showed severe blisters and erosions on multiple mucous membranes (i.e. lip, oral cavity, nose, eye, genitalia and anus) and on both the periungual and subungual regions. This is the first report in which an immunoblot analysis revealed the unusual combination of autoantibodies to both the p200 antigen and the gamma 2 subunit of laminin 5.

译文

Anti-p200天疱疮是一种自身免疫性表皮下水疱性疾病,其特征是针对下部lucina lucida的未知200 kDa酸性非胶原糖蛋白的自身抗体,而antilaminin 5粘膜天疱疮的特征是针对主要基底膜细胞外基质laminin 5的自身抗体。我们报道了一名64岁的日本女性,患有10年后的卵巢透明细胞癌淋巴结转移相关的表皮下水疱性疾病。临床表现为在多个粘膜(即嘴唇,口腔,鼻子,眼睛,生殖器和肛门)以及在唇周和舌下区域均出现严重的水泡和侵蚀。这是第一份免疫印迹分析显示针对p200抗原和层粘连蛋白5的gamma 2亚基的自身抗体异常组合的报告。

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