Extramammary Paget's disease of the perineum: Avoiding pitfalls in diagnosis and management.

Extramammary Paget's disease (EMPD) is a rare entity, especially in the perinoscrotal region, and typically presents in elderly white patients as a pruritic white or red patch in the area of distribution of apocrine glands. Typically, it affects a single site. Since its manifestations are insidious and easily misdiagnosed, the appropriate management is delayed. Management of this problem is complex and effective treatment can not only lower recurrence rates but also provide an optimal reconstructive result. The present report describes three patients with scrotal EMPD. Based on literature search, the etiopathology, diagnosis and management of these lesions is discussed. Reconstructive options, with special emphasis on scrotal lesions, are also discussed.