副肿瘤性天疱疮并发盆腔内Castleman病

Paraneoplastic pemphigus complicated with Castleman's disease in the pelvic cavity: one case report

报告1例副肿瘤天疱疮并发儿童型Castleman病。患者男,15岁。黏膜糜烂5个月余,全身红斑、水疱伴发热4个月。皮肤科检查:全身皮肤泛发红斑、斑丘疹,大小不一的厚壁水疱,不易破溃,尼氏征阴性。黏膜糜烂、溃疡,表面附着脓性分泌物。皮损组织病理及间接免疫荧光支持副肿瘤性天疱疮诊断,盆腔CT提示左侧髂外动脉、闭孔旁单发结节和肿物,切除肿物经组织病理证实为Castleman病。术后15 d患者死于感染性休克。

A case of paraneoplastic pemphigus complicated with Castleman＇s disease is reported. A 15-year-old boy present- ed with mucosal erosion for over 5 months, and erythema and blisters over the whole body as well as fever for 4 months. Physical examination showed the diffuse erythemas and maculopapules all over the body, not easily broken blisters with thick- ened wall and various sizes, negative Nokolsky＇s sign, and mucosal erosion and ulceration with purulent secretions on the sur- face. The diagnosis of paraneoplastic pemphigus was confirmed by histopathological examination and indirect immunofluorescenee. The CT scan of pelvic cavity showed single nodules on the left external iliac and obturator. The histopathological characters of the nodule confirmed the diagnosis of Castleman＇s disease. The patient died from septic shock 15 days after operation.