Nishibori Y, Hashimoto T, Ishiko A, Shimizu H, Korman N J, Nishikawa T
Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.
Dermatology. 1995;191(1):39-42. doi: 10.1159/000246485.
Paraneoplastic pemphigus is a rare skin disease characterized by painful mucosal ulcerations and polymorphous skin lesions in association with an underlying neoplasm. A 54-year-old Japanese man, who had received chemotherapy and radiation therapy due to a diagnosis of non-Hodgkin's lymphoma, developed marked stomatitis, conjunctivitis and blisters. Histologic examination showed suprabasal cleft formation with acantholysis and keratinocyte necrosis. Direct immunofluorescence revealed the deposition of IgG at the cell surface of the keratinocytes and C3 at the basement membrane zone. Indirect immunofluorescence on normal human skin and rat bladder sections revealed circulating autoantibodies to the cell surface of both keratinocytes and transitional epithelia. Immunoprecipitation disclosed antibodies reactive to the 250-kD, 230-kD, 210-kD and 170-kD proteins. From these results, a diagnosis of paraneoplastic pemphigus was made. This is the first report of paraneoplastic pemphigus from Japan.
副肿瘤性天疱疮是一种罕见的皮肤病，其特征为疼痛性黏膜溃疡和多形性皮肤损害，并伴有潜在的肿瘤。一名54岁的日本男性，因诊断为非霍奇金淋巴瘤接受了化疗和放疗，之后出现了明显的口腔炎、结膜炎和水疱。组织学检查显示基底层上裂隙形成伴棘层松解和角质形成细胞坏死。直接免疫荧光显示IgG沉积于角质形成细胞表面，C3沉积于基底膜带。对正常人皮肤和大鼠膀胱切片进行间接免疫荧光检查发现，循环自身抗体针对角质形成细胞和移行上皮细胞表面。免疫沉淀法显示抗体与250-kD、230-kD、210-kD和170-kD蛋白反应。根据这些结果，诊断为副肿瘤性天疱疮。这是来自日本的副肿瘤性天疱疮的首例报告。
