Extramammary Paget's disease: Report of two cases located on the vulva and penis

Abstract

Extramammary Paget's disease (EMPD) is a rare intraepithelial adenocarcinoma involving the epidermis and sometimes extends into the dermis. EMPD is most commonly seen in the intraepidermal primary form and rarely it occurs as a secondary form associated with malignancies. EMPD mostly occurs in areas such as vulva, penis, scrotum, perineum and axilla where apocrine glands are located. The most common site of involvement is vulva (65%). EMPD of male genital organs is less common, it accounts for 14% of cases. EMPD is a rare entity with very limited data in the literature and EMPD may occur in association with an underlying malignancy in adjacent organs. The diagnosis of EMPD is often delayed because of its non-specific features, its rarity, as well as the use of various treatments for dermatitis. The diagnosis and clinical management of EMPD is difficult. Biopsy should be taken from genital lesions unresponsive to treatment and histopathological examination, which is the basis of the diagnosis, should be performed. A detailed examination is necessary to detect an associated malignancy after diagnosis. Long-term follow-up of patients for regional lymphadenopathy, distant metastasis, local recurrence and development of internal malignancy form the basis of clinical management.

Keywords

• extramammary
• genital
• paget
• penis
• vulva