在这篇关于恶性肿瘤相关性天疱疮/恶性肿瘤相关性自身免疫多器官综合征（PNP/PAMS）的继续医学教育文章的第二部分中，将对其诊断标准、调查工作和治疗进行回顾。PNP/PAMS是一种与高发病率和高病死率相关的罕见自身免疫性水疱性疾病。识别PNP/PAMS的关键特征和其诊断标准对于开始适当的调查工作至关重要。评估PNP/PAMS需要了解其在组织病理学、直接免疫荧光、间接免疫荧光和酶联免疫吸附法上的发现。最后，本文回顾了PNP/PAMS的治疗方法，并根据文献中的病例报告和专家意见提出了建议。学习目标：完成此学习目标后，读者将能够确定诊断恶性肿瘤相关性天疱疮（PNP/PAMS）所需的标准，学会如何进行PNP/PAMS的诊断工作，并了解PNP/PAMS管理中的重要原则。版权所有 © 2023 Elsevier Inc. 发表

In the second part of this Continuing Medical Education article on paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome (PNP/PAMS), its diagnostic criteria, investigative work-up, and management are reviewed. PNP/PAMS is a rare autoimmune blistering disorder associated with high morbidity and mortality. Recognizing PNP/PAMS's key features and its diagnostic criteria is critical in initiating appropriate work-up. Evaluating PNP/PAMS requires knowledge of its findings on histopathology, direct immunofluorescence, indirect immunofluorescence, and enzyme-linked immunosorbent assay. Lastly, treatments for PNP/PAMS are reviewed with suggestions based on case reports and expert opinions in the literature. LEARNING OBJECTIVES: After completing this learning objective, the reader will be able to identify the criteria necessary for diagnosing paraneoplastic pemphigus (PNP/PAMS), learn how to work-up a diagnosis of PNP/PAMS, and understand important principles in the management of PNP/PAMS.Copyright © 2023. Published by Elsevier Inc.