Perianal Paget's disease: an isolated extramammary manifestation of Paget's

Perianal Paget's disease (PPD) is a rare condition which is often associated with underlying malignancy. To definitively rule out malignancy, excision biopsy is required. Surgery is the mainstay of treatment for this condition. A male patient in his 70s was referred to the colorectal outpatient clinic by a dermatologist with a punch biopsy result demonstrating PPD. His only presenting complaint was pruritus ani. Repeat biopsies confirmed the diagnosis of PPD and flexible sigmoidoscopy excluded luminal pathology. With complete preoperative work-up, a wide local excision was performed with a concurrent defunctioning colostomy to reduce local contamination and facilitate healing of delayed closure with both VY and mucosal advancement flaps. Delayed closure allows time to confirm histology results prior to definitive closure. We conclude that PPD is a rare condition which requires extensive and complete preoperative workup to diagnose underlying malignancy, which has significant implications on treatment and prognosis.