癌性天疱疮（PNP）/癌性自身免疫多器官综合征（PAMS）是一种高度致命的自身免疫性水疱性疾病。该病症发生在患有良性或恶性肿瘤的患者中，其中淋巴增生性疾病最为常见。体液免疫和细胞介导的免疫均参与了病因的发生，而针对角蛋白家族蛋白的自身抗体则是其特征。患者通常表现为严重口腔炎和多形性皮损，治疗常常难以奏效。支气管腺病（BO）是一种常见并发症，增加了PNP/PAMS的高死亡率。鉴于这种疾病的少见性和临床表现的异质性，临床医生在疑诊PNP/PAMS时应保持较高的警惕性，以避免延误诊断。本系列连续医学教育（CME）的第一部分将讨论PNP/PAMS的危险因素、病因以及临床特征。版权所有 © 2023 Elsevier Inc.

Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome (PNP/PAMS) is a highly fatal autoimmune blistering disease. The condition occurs in patients with underlying benign or malignant neoplasms, most commonly lymphoproliferative disorders. Both humoral and cell-mediated immunities contribute to the pathogenesis, and autoantibodies against plakin family proteins are characteristic. Patients typically present with severe stomatitis and polymorphous skin lesions, which are often resistant to treatment. Bronchiolitis obliterans (BO) is a frequent complication which contributes to the high mortality rate of PNP/PAMS. Given the rarity of this disorder and heterogeneity of clinical presentation, clinicians should maintain a high index of suspicion for PNP/PAMS to avoid delayed diagnosis. In this first part of a two-part continuing medical education (CME) series, risk factors, pathogenesis, and clinical features of PNP/PAMS are discussed.Copyright © 2023. Published by Elsevier Inc.