与复发性Castleman病相关的副肿瘤天疱疮1例

A Case of Paraneoplastic Pemphigus Associated with Recurrent Castleman's Disease

报告 1例与复发性Castleman病相关的副肿瘤天疱疮。患者男 ,2 9岁。主因进行性加重的疼痛性粘膜损害 4个月 ,躯干部多形性皮疹 5天入院。 7年前患者切除一腹膜后Castleman肿瘤 ,此次入院检查发现右腹膜后肿物 ,手术切除并经病理证实为Castleman肿瘤。患者临床、病理及免疫学特点均符合副肿瘤天疱疮。经大剂量静滴丙种球蛋白及口服强的松联合治疗 ,术后 7周皮疹完全消退 ,留下广泛色素沉着 ,术后

A case of paraneoplastic pemphigus associated with recurrent Castleman's disease is reported.The 29 year old man suffered from progressive painful mucosal erosions for four monthes and polymorphous skin lesion all over the body for 5 days.A Castleman's tumor was removed from his retroperitoneum.After this admission,a Castleman's tumor was detected and excised.With the administration of high dose intravenous immunoglobulin and prednisone,complete remission occurred 9 weeks after tumor resection,leaving extensive melanosis.