August 14, 2025Open Access

Pigmented Extramammary Paget’s Disease of the Axilla: Two Case Reports and a Literature Review

Key Points

Two young women with pigmented extramammary Paget's disease were diagnosed using immunohistochemical analysis, revealing a rare condition that mimics other skin lesions.
Both cases presented in the axilla, with one patient being the youngest reported at 31 years old, highlighting the condition's rarity in such demographics.
Mohs micrographic surgery was performed on both patients, resulting in no recurrence or metastasis postoperatively, showcasing a positive outcome.
This report emphasizes the importance of accurate diagnosis to differentiate extramammary Paget’s disease from similar appearing skin conditions.

Abstract

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.

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