腋窝非乳头皮肤Paget病（EMPD）是一种罕见疾病，文献中只报告了少数病例。我们进行了回顾性研究，发现16例腋窝受累的EMPD病例。我们总结了临床和组织病理学特征、治疗和预后，以及回顾了文献。包括八名男性和八名女性在内的患者，平均诊断年龄为63.9岁。11例患者表现为单侧腋窝病变，2例患者表现为双侧腋窝病变，3例患者表现为腋窝和生殖器官受累。四名男性患者有继发性恶性肿瘤的病史。腋窝EMPD呈现出Paget病的典型组织学和免疫组化特征。除一例外，所有患者均接受莫氏显微手术，最终平均边缘为1.3厘米，肿瘤以1厘米边缘清除率为76.5%。随访平均63.6个月后，没有患者出现复发或转移。腋窝EMPD与典型EMPD具有相似的临床病理特征。必须仔细进行临床和病理学检查，以检测可能存在的相关恶性肿瘤并进行正确的诊断。腋窝EMPD通常有良好的预后。由于EMPD总体的完整边缘评估和更好的复发率，莫氏显微手术是首选治疗方法。 ©2023国际皮肤科学会。

Axillary extramammary Paget's disease (EMPD) is a rare condition with only a few cases reported in the literature.We performed a retrospective review and identified 16 cases of EMPD with axillary involvement. We summarized the clinical and histopathological characteristics, treatment, and prognosis, as well as reviewed the literature.Of the included patients, eight were male and eight were female with an average age of 63.9 years at diagnosis. Eleven patients presented with unilateral axillary lesions, two patients with bilateral axillary lesions, and three patients with both axillary and genital involvement. Four male patients had a history of secondary malignancies. Axillary EMPD exerted the typical histological and immunohistochemical features of Paget's disease. All except for one patient underwent Mohs micrographic surgery with a mean final margin of 1.3 cm, and the tumor was cleared 76.5% of the time with 1 cm margins. None of the patients developed recurrence or metastasis after surgery at a mean follow-up of 63.6 months.Axillary EMPD shares similar clinicopathological features with typical EMPD. Careful clinical and pathological examinations are mandatory to detect possible associated malignancies and to make a correct diagnosis. Axillary EMPD usually has a good prognosis. Due to the complete margin assessment and better recurrence rates for EMPD in general, Mohs micrographic surgery is the treatment of choice.© 2023 the International Society of Dermatology.