Extramammary Paget's disease

EMPD is a rare condition that poses difficulties of diagnosis and management. Suspicious skin lesions not responding to topical therapy after four to six weeks should be biopsied to exclude EMPD. There is an associated malignancy in 20% to 30% of cases and a detailed investigation of the patient should be carried out at presentation to exclude invasive disease. Surgery remains the mainstay of treatment for EMPD but carries a 40% local recurrence rate. As a result of this, other treatment modalities have been used mostly on an experimental basis to treat EMPD, including radiotherapy, topical and systemic chemotherapy, Mohs' micrographic surgery (MMS), laser therapy and PDT. The British Society for the Study of Vulval Disease (http://www.bssvd.fsnet.co. uk) has established a national register of cases and patients may benefit from referral to centres of expertise in treating vulval conditions. Because EMPD is so rare, there is currently a lack of appropriately controlled clinical trials comparing the various methods of treatment and the precise role of each modality of treatment in the management of this condition has yet to be elucidated. Nevertheless, it is clear that follow up of treated EMPD must be long term whatever the primary mode of treatment, because of the propensity of this condition to recur. Multicentre randomised controlled trials are required to compare different treatments. © RCOG 2004.