目的：研究副肿瘤天疱疮（PNP）/副肿瘤性自身免疫多器官综合征（PAMS）的临床特点。方法回顾性总结我院2013年7月至2004年1月收治的16例PNP/PAMS患者的临床表现、组织病理及免疫学特征、治疗及预后情况。结果多形性皮肤黏膜损害为其主要临床表现，7例患者存在闭塞性细支气管炎或呼吸衰竭。组织病理显示，基底层上棘层松解、基底细胞液化变性及角化形成细胞坏死。直接及间接免疫荧光显示，自身抗体沉积于棘细胞间和（或）基底膜带。15例患者伴发血液系统肿瘤，其中Castleman病最常见。大剂量糖皮质激素联合免疫调节剂对控制症状有一定作用，而手术切除肿瘤与疾病转归相关。结论 PNP/PAMS具有特殊的临床、组织病理及免疫学表现，早期诊断、手术切除肿瘤、联合糖皮质激素等药物是治疗PNP/PAMS的关键。%Objective To investigate the clinical features of paraneoplastic pemphigus(PNP) or paraneoplastic au-toimmune multiorgan syndrome(PAMS). Method 16 patients with PNP/PAMS treated in our hospital from July 2013 to January 2014 were analyzed for clinical manifestations, histopathological and immunological features, treat-ment and prognostic outcomes, retrospectively. Result Polymorphous mucocutaneous eruption were the hallmark of PNP/PAMS. Respiratory failure or bronchiolitis obliterans were observed in 7 patients. The most common histologi-cal features were suprabasal acantholysis, vacuolar degeneration of the basal cell layer and keratinocyte necrosis. Au-toantibodies deposition at the extracellular space or along the basement membrane zone were found by the direct and indirect immunofluorescence. Hematological neoplasms were observed in 15 patients, with Castleman ’s disease as the most frequent settings. High-dose corticosteroids in conjunction with other immunomodulators could alleviate symptoms, but a better prognosis was associated with tumor resection and Castleman ’s disease. Conclusion Early diagnosis, complete removal of tumor, and therapeutic modalities including high-dose corticosteroids and immuno-modulators were essential to PNP/PAMS treatment.
展开▼
