Extra-mammary Paget's disease is a rare cancer affecting the anogenital region and can present with a myriad of symptoms. In women, the diagnosis of Paget's disease can be challenging as it mimics several other vulval conditions. It is important to promptly recognise this disease because of its potential association with synchronous tumours, such as colorectal adenocarcinoma. The mainstay of treatment is with immunomodulating therapies or surgery, but unfortunately the risk of recurrence is high and appears to be independent of treatment choice and tissue margin status. There is growing evidence to move away from traditional surgical excision to using topical therapy, such as imiquimod, as surgery can often be extensive and disfiguring with a prolonged recovery time. There is considerable psychosexual and physical morbidity associated with Paget's disease, largely owing to multiple surgical recurrences. As recurrences can occur several years after the initial presentation, long-term follow up of patients is recommended.
Keywords: Extra-mammary Paget's disease; Imiquimod; Surgical excision of Paget's disease; Vulval Paget's disease.