Extramammary Paget's disease (EMPD) is a rare malignancy of skin. It primarily affects apocrine gland-bearing skin areas such as vulva, scrotum, and penis. It presents with vague dermatological symptoms at initial presentation and has a long latency period before evidence of cancer or metastasis appears. Patient GS, 72 year-old Filipino male, with no comorbidities, unremarkable exposure or sexual history came in with a 7 year history of an enlarging erythematous lesion at the left scrotum, associated with pruritus. Multiple consultations with a dermatologist, and therapy with multiple oral and topical antihistamines, antibacterials, antifungals and steroids did not resolve the lesion. Persistence prompted urology consult.

Incision biopsy of lesion was done with a 1 cm margin. Histopathology revealed Extramammary Paget’s Disease of the Scrotum. Patient underwent chest and whole abdominal CT scan to assess for metastasis, negative results. Cystoscopy was done, unremarkable. Patient underwent wide local excision of the scrotal lesion with rush frozen section.

Patient was referred to plastic surgery for scrotal reconstruction. Patient underwent V-Y advancement flap of the skin overlying the gracilis and vastus medialis muscles, with advancement to the circumferential defect at the base of the penis Post operative course was unremarkable.

There are no definitive guidelines outlining the management of EMPD. Surgical excision with negative margins is the mainstay treatment for EMPD.