Beyond the Ordinary: A Surgical Approach to Perianal Paget Disease

Abstract Perianal Paget Disease (PPD) is a rare form of extramammary Paget disease characterized by erythematous or eczematous plaques in the perianal region. Its rarity and resemblance to benign skin conditions often result in delayed diagnosis. We present the case of a 56-year-old woman with a 4-year history of a refractory perianal lesion. A sharply demarcated erythematous plaque was identified, and biopsy confirmed PPD. Further workup excluded any underlying malignancy. Wide local excision with a 10-mm margin was performed, sparing the anal sphincters, followed by immediate reconstruction using a right gluteal fasciocutaneous V-Y advancement flap. The postoperative period was uneventful, and pathology confirmed negative margins. Perianal Paget Disease requires differentiation from benign skin conditions and thorough evaluation for underlying malignancies. Many treatment options are available. However, surgery—more specifically wide local excision—remains the standard treatment. Despite the lack of standardized guidelines, long-term follow-up is essential due to the risk of recurrence and malignancy. The present report highlights the importance of considering PPD in cases of chronic refractory perianal lesions and the need for developing guidelines for managing patients with PPD.