Unilateral Pigmented Extramammary Paget¡¯s Disease of the Axilla Associated with a Benign Mole: A Case Study and a Review of Literature

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(Ladak Aleem) - North Manchester General Hospital Breast Unit
(Bramley Maria) - North Manchester General Hospital Breast Unit
(Titi Sami) - Royal Oldham Hospital Department of Pathology

Abstract

Pigmented extramammary Paget¡¯s disease (PEMPD) is an uncommon intraepithelial adenocarcinoma and a rare variant of Paget¡¯s disease affecting skin that is rich in apocrine sweat glands such as the axilla, perianal region and vulva. It most commonly occurs in postmenopausal women and presents as a superficial pigmented scaly macule, mimicking a melanocytic lesion. The histological presentation is adenocarcinoma in situ with an increased number of melanocytes scattered between the Paget¡¯s cells. Therefore, PEMPD may be misdiagnosed as a melanocytic tumour both clinically and histologically. The tumour cells are usually positive for cytokeratin 7, epithelial membrane antigen, Cam 5.2, HER2, and mucicarmine stain while S100 and human melanoma black-45 highlight the processes of reactive dendritic cells. The association between Paget¡¯s cells and intratumoural reactive melanocytes is still unclear. We report our first case of PEMPD associated with an intradermal naevus involving the axilla in a 63-year-old woman.

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