a Department of Dermatology, Graduate School of Medical Sciences , Kyushu University , Fukuoka , Japan.
Expert Rev Anticancer Ther. 2018 Jun;18(6):543-553. doi: 10.1080/14737140.2018.1457955. Epub 2018 Mar 27.
Extramammary Paget's disease (EMPD) is a rare neoplastic condition that commonly affects the anogenital area in the elderly. Owing to its low incidence, limited data regarding EMPD's diagnosis and treatment have been available. This review article aims to explore the current knowledge of EMPD to improve the management of this disease. Areas covered: This review outlines the diagnosis and management of EMPD. Articles on this issue that had been published in PubMed were identified and surveyed. We provide an overview of the reported studies, focusing on the recent advances in this field. Expert commentary: A new TNM staging system specific for EMPD has been proposed in Japan; the T category was classified by tumor thickness and lymphovascular invasion, the N category by the number of metastatic lymph nodes, and the M category by systemic metastases. As new diagnostic tools for EMPD, dermoscopy and reflectance confocal microscopy have emerged. Recent reports about Mohs micrographic surgery, mapping biopsy, radiation therapy, photodynamic therapy, topical imiquimod, conventional chemotherapy, and targeted therapy are also discussed. Despite the increasing publications of EMPD, limited information on this condition is available and the accumulation of more data is required.
派杰氏病（EMPD）是一种罕见的肿瘤性疾病，常见于老年人群的会阴部。由于其发病率低，有关 EMPD 的诊断和治疗的数据有限。本文旨在探讨 EMPD 的现有知识，以改善该疾病的管理。
本文概述了 EMPD 的诊断和治疗。在 PubMed 上确定并调查了关于这个问题的文章。我们提供了报告研究的概述，重点关注该领域的最新进展。
日本提出了一种新的 EMPD 特定的 TNM 分期系统；T 类根据肿瘤厚度和脉管侵犯进行分类，N 类根据转移淋巴结的数量，M 类根据全身转移。作为 EMPD 的新诊断工具，出现了皮肤镜和反射共聚焦显微镜。最近关于 Mohs 显微外科手术、绘图活检、放射治疗、光动力疗法、局部咪喹莫特、常规化疗和靶向治疗的报告也进行了讨论。尽管 EMPD 的出版物越来越多，但关于这种疾病的信息有限，需要积累更多的数据。
