Extramammary Paget's disease (EMPD) is a rare cutaneous malignancy. The disease typically affects older individuals aged 60-80 years and is seen most frequently in postmenopausal Caucasian women and Asian men. EMPD exhibits a predilection for the genital and perianal regions and may be associated with an underlying carcinoma in adjacent organs. EMPD presents a challenge in both diagnosis and management. Often treated empirically as various dermatitides, the correct diagnosis is frequently delayed by many years. Following diagnosis, an extensive search for an associated malignancy should be initiated. If invasive disease is present on biopsy, a sentinel lymph node biopsy may guide further treatment. Mohs micrographic surgery appears to be superior to wide local excision when considering tissue sparing ability and disease recurrence. Nonsurgical interventions have also been investigated with varied results. Regardless of treatment method, long-term follow-up is recommended to monitor for local disease recurrence, development of internal malignancy, regional lymphadenopathy, or distant metastasis.
© 2018 The International Society of Dermatology.