Wang Yi-Xiao, Cui Xiao-Mei, Zhang Qing, Xu Xiao-Qing, Sun Yao, Gao Min, Gu Li-Xiong
Department of Dermatology, The First Affiliated Hospital of Anhui Medical University, Hefei, Anhui, 230032, People's Republic of China.
Institute of Dermatology, Anhui Medical University, Hefei, Anhui, 230032, People's Republic of China.
Clin Cosmet Investig Dermatol. 2025 Aug 8;18:1909-1915. doi: 10.2147/CCID.S535004. eCollection 2025.
Extramammary Paget's disease is a rare intraepithelial adenocarcinoma that mainly occurs in areas rich in apocrine sweat glands, such as the vulva, perianal region, and, less commonly, in the axillary skin. Owing to the varied manifestations of extramammary Paget's disease, it is often misdiagnosed. Particularly, pigmented extramammary Paget's disease (PEMPD) can be mistaken for superficial spreading melanoma, Bowen's disease, pigmented superficial basal cell carcinoma, or seborrheic keratosis. Here, we report two cases of women with axillary pigmented plaques who were confirmed as PEMPD through histopathological and immunohistochemical analyses. One of these patients was only 31 years old, which makes her the youngest reported case of PEMPD to date. All patients underwent Mohs micrographic surgery, and no recurrence or metastasis was observed postoperatively. PEMPD is a rare clinical variant that can resemble other pigmented lesions, both clinically and histopathologically. Therefore, this report underscores the importance of accurate differential diagnosis through immunohistochemistry.
乳腺外佩吉特病是一种罕见的上皮内腺癌，主要发生在富含顶泌汗腺的区域，如外阴、肛周区域，较少见于腋窝皮肤。由于乳腺外佩吉特病表现多样，常被误诊。特别是色素沉着性乳腺外佩吉特病（PEMPD）可被误诊为浅表扩散性黑色素瘤、鲍温病、色素性浅表基底细胞癌或脂溢性角化病。在此，我们报告两例腋窝色素沉着斑块的女性患者，经组织病理学和免疫组化分析确诊为PEMPD。其中一名患者年仅31岁，是迄今为止报道的最年轻的PEMPD病例。所有患者均接受了莫氏显微外科手术，术后未观察到复发或转移。PEMPD是一种罕见的临床变异型，在临床和组织病理学上都可能类似于其他色素性病变。因此，本报告强调了通过免疫组化进行准确鉴别诊断的重要性。
