Management of Extramammary Paget's Disease: A Case Report and Review of the Literature

Extramammary Paget's Disease (EMPD) is a rare condition of the skin that often involves the vulva, perianal region, scrotum, penis, and axilla. Although prognosis is generally favorable, it can be associated with neoplasms of the bladder, urethra, prostate, and rectum. This report presents a case of scrotal EMPD that failed treatment with imiquimod 5% cream and discusses benefits and complications of available treatment options. The variation of treatment success emphasizes the importance of further research. 1. Introduction Extramammary Paget’s Disease (EMPD) is a rare neoplastic condition of the skin or its underlying appendages commonly found in the vulva, perianal region, scrotum, penis, and axilla [1]. Clinically the condition presents as a well-demarcated, thickened, pruritic, erythematous, or white scaly plaque with irregular borders [2, 3]. Microscopically EMPD involves large cells with vacuolated cytoplasm and centrally located nuclei characterized as Paget cells [4]. The disease is categorized into primary or secondary EMPD with primary EMPD originating from intraepidermal cells and secondary EMPD coming from an underlying neoplasm [1, 4]. Neoplasms of the bladder, urethra, and prostate are associated with EMPD involving the external genitalia, while rectal adenocarcinoma is associated with perianal EMPD [5, 6]. The true incidence is still unclear but Karam and Dorigo found the median age at diagnosis to be 72 years with EMPD predominately occurring in Caucasians and women having a higher occurrence than men. Prognosis is generally favorable; however, older age, advance stage, and treatment modality can be associated with worse outcomes [1]. Although surgery is not always a feasible option for patients, surgical resection with clear margins is considered to be the standard of care. Other treatment options for EMPD involve, imiquimod 5% topical cream, modified peripheral Mohs surgery, and radiation therapy [1–3, 7, 8]. Many case studies have reported successful outcomes with 5% imiquimod cream in patients who did not undergo surgical treatment. In this paper, we present a case of scrotal EMPD that failed treatment with imiquimod 5% cream and discuss the benefits and complications of other treatment plans. 2. Case Report A 73-year-old white male presented with a 2-year history of a pruritic, erythematous lesion over his scrotum. Physical examination revealed an erythematous plaque extending over the right and left scrotum with an uninvolved, 1.5？cm strip at the median raphe for a distance of 7？mm on either side. The involved area contained