副肿瘤性天疱疮1例  

Paraneoplastic pemphigus: a case report

作　　者：曹冰青[1,2] 马东来[1] 李红春[1] 李丽[1] 王宝玺[1] 

机构地区：[1]中国医学科学院,中国协和医科大学北京协和医院皮肤科 [2]河南安阳地区医院皮肤科,455000

出　　处：《临床皮肤科杂志》2005年第5期305-307,共3页Journal of Clinical Dermatology

摘　　要：报告1例副肿瘤性天疱疮。患者男,31岁。因口腔黏膜疼痛性、难治性糜烂6个月,全身皮肤红斑、丘疹,伴脱发1个月就诊。体格检查:头部呈虫蚀状及斑片状脱发,口唇及外阴黏膜弥漫性肿胀、糜烂,全身皮肤散在多形性红斑、丘疹,偶见水疱,手、足部可见点状或片状水肿性红斑伴鳞屑。增强CT检查示右肾上腺区肿物,考虑Castleman瘤。外科手术切除一9cm×6cm大肿瘤,组织病理检查示透明血管型巨大淋巴结增生,伴局灶钙化;免疫组化CD3(+)、CD20(+)、CD21(+)、CD68(+)。诊断为副肿瘤性天疱疮。术后应用糖皮质激素治疗皮损好转。A 31-year-old man was admitted for recurrent painful erosive lesions on his lips for six months, and erythema, papules occurred on the trunk and extremities and hair loss for one month. Physical examination revealed scattered erythema multiforme-like lesions, papules and sporadic vesicles all over the body. Erythematous edema, erosions and crusts could be found on the lips, glans penis and scrotum. Scaly erythema was presented on his hands and feet. The contrast CT showed there was a Castleman tumor at the right adrenal glands site. A tumor about 9 cm × 6 cm size was excised by surgical operation. Histopathology confirmed the giant lymphadenovarix to be Castleman tumor. Immunopathology revealed that lymphocyte markers such as CD3, CD20, CD21, CD68 were all positive. The diagnosis of paraneoplastic pemphigus was then made.

关 键 词：天疱疮 副肿瘤性 瘤 Castleman 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]