伴限局性Castleman病的副肿瘤性天疱疮1例  

A case of paraneoplastic pemphigus with localized Castleman's disease

作　　者：陈翔[1] 田立红[1] 陈服文[1] 杜乾君[1] 谢红付[1] 林伟[1] 林静[1] 树叶[1] 颜克香[1] 

机构地区：[1]中南大学湘雅医院皮肤科,湖南长沙410008

出　　处：《临床皮肤科杂志》2006年第3期166-167,共2页Journal of Clinical Dermatology

摘　　要：报告1例伴限局性Castleman病的副肿瘤性天疱疮(paraneoplasticpemphigus,PNP)及其电镜所见。患者女,33岁。口腔黏膜糜烂3个月,外阴溃疡2个月,面部、躯干及四肢出现多形性皮损20余天。影像学检查发现腹膜后实质性肿块,手术切除肿块后经组织病理证实为血管滤泡性淋巴结增生。皮损组织病理检查符合PNP改变。间接免疫荧光显示棘细胞间IgG沉积。电镜下见基底层上棘细胞松解及凋亡。腹部肿块切除后予糖皮质激素和对症治疗,皮损逐渐消退。A case of paraneoplastic pemphigus （PNP） with localized Castleman＇s disease is reported. A 33-year-old female presented painful erosions on the lips and oral mucosa for 3 months and ulcers of vulva for 2 months and multiform-like lesions for 20 days. Histopathology showed that the tumour discovered through imageology was Castleman＇s disease after exairesis. Histopathology of the cutaneous lesions showed PNP. Indirect immunofluorescence demonstrated the present of IgG de posited among prickle cells. Electron microscope demonstrated keratinocyte apoptosis and acantholytic cell over basal layer.

关 键 词：天疱疮 副肿瘤性 CASTLEMAN病 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]