Extramammary Paget disease (EMPD) is a rare malignancy that primarily arises in apocrine gland-bearing areas such as the genital, perianal, and axillary regions. The clinical and biological heterogeneity of EMPD, together with a lack of evidence from large-scale studies, has made it difficult to establish standardized approaches to diagnosis and treatment. To address these issues, the 2025 Japanese Guidelines for the Management of EMPD were developed, providing evidence-based recommendations tailored to the healthcare context in Japan. These guidelines were constructed using the Minds Guideline Development Manual 2020 (ver. 3.0), adhering to systematic review principles and incorporating both Japan and international research findings. A multidisciplinary panel of dermatologists, oncologists, surgeons, and other specialists developed the guidelines through a consensus-based process, which included structured discussions and grading of evidence. The primary focus of this condensed version is on six critical clinical questions, which address key aspects of EMPD management, including the role of mapping biopsy in diagnosis, the use of sentinel lymph node biopsy in cases of suspected dermal invasion, the efficacy of nonsurgical therapies when surgery is not an option, the efficacy of lymph node dissection for multiple regional lymph node metastases, the efficacy of radiation therapy after regional lymph node dissection, and systemic treatment options for advanced disease. The recommendations aim to improve diagnostic accuracy, optimize therapeutic approaches, and support clinical decision-making by providing a clear framework for the management of EMPD. By focusing on these critical areas, the guidelines strive to enhance patient outcomes and contribute to the advancement of evidence-based care for this rare and challenging malignancy.

乳房外Paget病（EMPD）是一种罕见恶性肿瘤，主要发生于顶泌腺分布区域，如生殖器、肛周及腋窝。EMPD的临床和生物学异质性，加之缺乏大规模研究证据，导致难以建立标准化的诊疗方案。为解决这些问题，《2025年日本EMPD管理指南》应运而生，该指南基于循证医学，结合日本医疗实际情况提供推荐意见。指南的制定遵循《Minds指南制定手册2020》（3.0版），秉持系统综述原则，整合了日本及国际研究成果。由皮肤科医生、肿瘤科医生、外科医生及其他专科医生组成的多学科专家组，通过基于共识的流程（包括结构化讨论和证据分级）完成了指南制定。

本精简版指南重点关注六个关键临床问题，涉及EMPD管理的核心方面：包括Mapping活检在诊断中的作用、疑似真皮浸润时前哨淋巴结活检的应用、无法手术时非手术治疗的疗效、多发性区域淋巴结转移时淋巴结清扫的疗效、区域淋巴结清扫后放射治疗的疗效，以及晚期疾病的系统治疗选择。这些推荐意见旨在提高诊断准确性、优化治疗方案，并通过提供清晰的EMPD管理框架为临床决策提供支持。通过聚焦这些关键领域，指南致力于改善患者预后，推动这一罕见且难治性恶性肿瘤的循证医疗发展。