IgA pemphigus with paraneoplastic pemphigus-like clinical features showing IgA antibodies to desmoglein 1/3 and desmocollin 3, and IgG and IgA antibodies to the basement membrane zone.
IgA pemphigus with paraneoplastic pemphigus-like clinical features showing IgA antibodies to desmoglein 1/3 and desmocollin 3, and IgG and IgA antibodies to the basement membrane zone.
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IgA 天疱疮具有副肿瘤性天疱疮样临床特征，显示针对桥粒芯糖蛋白 1/3 和桥粒胶蛋白 3 的 IgA 抗体，以及针对基底膜区的 IgG 和 IgA 抗体。
DOI：
10.1111/ced.12050
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发表时间：
2013
期刊：
影响因子：
--
通讯作者：
Hashimoto T.
中科院分区：
文献类型：
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作者：
Ueda A;Ishii N;Temporin K;Yamazaki R;Murakami F;Fukuda S;Hamada T;Dainichi T;Kyoya M;Saito C;Matsunaga R;Kimura S;Kawakami T;Soma Y;Hashimoto T.
一名79岁的日本妇女在她的口腔黏膜出现严重的顽固性糜烂，类似于副肿瘤天疱疮。采用间接免疫荧光法检测细胞表面IgA抗体，基底膜区IgG抗体和IgA抗体。免疫印迹显示IgG抗体与大疱性类天疱疮230和periplakin反应弱，而IgA抗体与任何抗原均无反应。采用ELISA法检测抗粘粒蛋白（Dsg）1和Dsg3的IgA抗体。用转染到COS‐7细胞的人Dsc1-3的cdna检测到desmocollin (Dsc)3的IgA抗体。尽管口服强的松龙、大剂量静脉注射免疫球蛋白和双滤过血浆置换治疗，皮肤病变仍然活跃，患者死于闭塞性细支气管炎样呼吸衰竭。尽管进行了广泛的调查和尸检，但没有发现潜在的肿瘤。复杂的免疫病理结果可能在患者异常临床特征的发展中发挥了重要作用。
A 79‐year‐old Japanese woman presented with severe recalcitrant erosions on her oral mucosa, resembling paraneoplastic pemphigus. Using indirect immunofluorescence, we detected IgA antibodies against the cell surface, and both IgG and IgA antibodies against the basement membrane zone. Immunoblotting showed that the IgG antibodies reacted weakly with bullous pemphigoid 230 and periplakin, whereas the IgA antibodies did not react with any antigen. IgA antibodies to both desmoglein (Dsg)1 and Dsg3 were detected by ELISA. IgA antibodies to desmocollin (Dsc)3 were also detected by using cDNAs for human Dsc1–3 transfected into COS‐7 cells. Despite treatment with oral prednisolone, high‐dose intravenous immunoglobulin and double‐filtration plasmapheresis, the skin lesions remained active, and the patient died from bronchiolitis obliterans‐like respiratory failure. Despite extensive investigations and postmortem examination, no underlying neoplasms were found. The complex immunopathological findings probably played an important role in the development of the patient's unusual clinical features.