伴恶性胸腺瘤和闭塞性细支气管炎的副肿瘤性天疱疮一例

A Case of Paraneoplastic Pemphigus with Malignant Thymoma and Obliterated Bronchiolitis

副肿瘤性天疱疮(PNP)是一种罕见的、伴发淋巴细胞增生性肿瘤的危重症自身免疫性大疱性疾病,临床表现为严重的口腔黏膜糜烂及多形性的皮肤损害。Castleman病、非霍奇金淋巴瘤、胸腺瘤、滤泡树突状肉瘤及慢性淋巴细胞性白血病是PNP常见的伴发肿瘤,然而伴发恶性胸腺瘤则较为罕见。本病预后极差,死亡率高,死亡原因通常包括严重的感染、潜在的恶性肿瘤及闭塞性细支气管炎。本文报道1例58岁女性反复口腔糜烂溃疡3年,全身红斑水疱10个月,呼吸困难3个月,经临床、影像学、组织病理学、免疫学检查最终诊断为伴恶性胸腺瘤及闭塞性细支气管炎的PNP,该患者在恶性胸腺瘤切除后3周死于呼吸衰竭。

Paraneoplastic pemphigus(PNP)is a rare and life⁃threatening autoimmune blistering disease characterized by severe mucosal erosions and polymorphous cutaneous eruptions associated with lymphoproliferative neoplasmas.Castleman􀆳s disease,non⁃Hodgkin􀆳s lymphoma,thymoma,follicular dendritic cell sarcoma and chronic lymphocytic leukemia are the commonly associated neoplasmas in PNP,whereas malignant thymoma is rare.The prognosis of PNP is poor due to severe infections during immunosuppressive treatment,underlying malignancies and bronchiolitis obliterans mediated by autoimmunity.A 58⁃year⁃old woman who experienced recurrent oral erosive ulcer for 3 years,systemic erythema for 10 months,and dyspnea for 3 months was diagnosed with PNP with malignant thymoma and obliterated bronchiolitis by clinical,radiological,histopathological,and immunological examinations.She died of respiratory failure 3 weeks after resection of the malignant thymoma.