Extramammary Paget's disease of scrotum treated with radiotherapy

Extramammary Paget's disease (EMPD) of the scrotum is extremely rare. Most cases are found on the vulva or anus. It is generally accepted that EMPD is associated with an underlying carcinoma in situ. The lesion is located deep in between the dermal to epidermal layer. EMPD must be differentiated from benign papulosquamous disease, squamous cell carcinoma and melanoma. It can be managed by local excision and reconstruction with a skin graft or skin flap. Radiation and topical chemotherapy have also been used as alternative treatment strategies and are effective for local control.