副肿瘤性天疱疮合并小结树突细胞肉瘤的桥粒核心糖蛋白1和3抗体效价  

Antibody titers to desmogleins 1 and 3 in a patient with paraneoplastic pemphigus associated with follicular dendritic cell sarcoma

作　　者：SeishimaM. OdaM. Oyama Z. 田中伟 

机构地区：[1]Department of Dermatology, Ogaki Municipal Hospital, Minaminokawa-cho4-86,Ogaki503-8502,Japan Dr.

出　　处：《世界核心医学期刊文摘（皮肤病学分册）》2005年第4期61-61,共1页Digest of the World Core Medical JOurnals:Dermatology

摘　　要：Background: Most paraneoplastic pemphigus (PNP) cases reported to date have been associated with lymphoproliferative neoplasms. Patients with PNP have autoantibodies against the plakin family (eg, envoplakin and periplakin). Antibodies against desmoglein 3 (Dsg3) and Dsg1, antigens for classic types of pemphigus, have also been reported to play an important role in the initial stage of PNP. Observations: We describe a patient with PNP associated with follicular dendritic cell sarcoma. Antibodies to envoplakin and periplakin were detected. When only mucosal lesions were observed at the early stage, the antibody to Dsg3 but not to Dsg1 was detected by enzyme-linked immunosorbent assay. After skin lesions appeared, antibodies to Dsg1 and Dsg3 were dete cted. These titers were elevated, with exacerbation of skin lesions. Although the patient received corticosteroid therapy, double-filtration plasmapheresis, and intravenous human immunoglobulin therapy after surgical resection of follicular dendritic cell sarcoma, she died of fungal infective lung embolisms. A direct immunofluorescence study of autopsy samples showed IgG deposition intheepidermisoftheskinandoralmucosalmembrane,butnot in the lungs and kidneys and follicular dendritic cell sarcoma of the para-aortic area. Conclusion: In this patient with PNP and follicular dendritic cell sarcoma, there was an association between the clinical phenotype and the anti-Dsg antibody profile, as seen in pemphigus vulgaris.Background: Most paraneoplastic pemphigus (PNP) cases reported to date have been associated with lymphoproliferative neoplasms. Patients with PNP have autoantibodies against the plakin family (eg, envoplakin and periplakin). Antibodies against desmoglein 3 (Dsg3) and Dsg1, antigens for classic types of pemphigus, have also been reported to play an important role in the initial stage of PNP. Observations: We describe a patient with PNP associated with follicular dendritic cell sarcoma. Antibodies to envoplakin and periplakin were detected. When only mucosal lesions were observed at the early stage, the antibody to Dsg3 but not to Dsg1 was detected by enzyme-linked immunosorbent assay. After skin lesions appeared, antibodies to Dsg1 and Dsg3 were dete cted. These titers were elevated, with exacerbation of skin lesions. Although the patient received corticosteroid therapy, double-filtration plasmapheresis, and intravenous human immunoglobulin therapy after surgical resection of follicular dendritic cell sarcoma, she died of fungal infective lung embolisms. A direct immunofluorescence study of autopsy samples showed IgG deposition intheepidermisoftheskinandoralmucosalmembrane,butnot in the lungs and kidneys and follicular dendritic cell sarcoma of the para-aortic area. Conclusion: In this patient with PNP and follicular dendritic cell sarcoma, there was an association between the clinical phenotype and the anti-Dsg antibody profile, as seen in pemphigus vulgaris.

关 键 词：肿瘤性 小结树突细胞 桥粒 酶联免疫吸附法 免疫荧光检查 黏膜损害 IgG 皮质类固醇 增殖性 血浆分离 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]