Vulvular Paget’s Disease: A Rare Case Report

Extramammary Paget’s Disease (EMPD) of the vulva is a rare intraepithelial adenocarcinoma, which mainly affects postmenopausal women and often mimics eczematous dermatitis, leading to delayed diagnosis. We report a ۶۳-year-old woman with a scar-like vulvar lesion presenting for five years, which showed progressive enlargement over the past four months. The lesion was located on the right labia majora and associated with discomfort and generalized pruritus. Pelvic contrast-enhanced MRI revealed a malignant mass involving the cutaneous and subcutaneous tissues of the left pubic region. Histopathological findings suggested EMPD, which was confirmed by immunohistochemical positivity for CK۷, EMA, CEA, and GATA-۳. The patient underwent wide local excision with ۳ cm margins of clinically normal-appearing skin. Early intervention is important to reduce complications and metastatic risk. Surgical excision remains the primary treatment, and long-term follow-up is essential due to the risk of recurrence and possible metastasis.

کلیدواژه ها:

Diagnosis ، Extramammary paget&rsquo ، s disease ، Recurrence ، Surgery ، Treatment ، Vulvar paget&rsquo ، s disease