Perianal Paget's disease-Report of a rare case.

INTRODUCTION Perianal extra-mammary Paget's disease is a rare skin disorder of unknown aetiology, which is frequently associated with malignancy. This case report draws attention to this rare condition and comments upon its diagnosis and treatment. PRESENTATION OF CASE A 64-year-old otherwise fit man, presented to us in 2006 with one-year-long history of perianal irritation. On examination there was an erythematous discoid skin lesion in the right perianal area. The lesion was excised with wide margins and the defect closed with a local transposition flap. Histology confirmed extra-mammary Paget's disease (EMPD) with a focus of invasion showing a well-differentiated mucinous adenocarcinoma. Adjuvant therapy was not advised. On follow-up in 2011, a small irregular skin lesion, well away from the previous excision site was noted on the left perianal area. Biopsies from this lesion confirmed EMPD with no focus of invasion. Once again wide local excision with closure using local transposition flap was undertaken. Long term follow up has been advised. DISCUSSION The optimal treatment for Perianal Paget's disease (PPD) remains controversial. Surgery is the commonest modality used with wide local excision being the treatment of choice for resectable disease. We report herein a short review of various therapies reported so far in the management of this rare disorder. CONCLUSION A thorough initial evaluation and long-term follow-up is essential to identify recurrence and the development of other related malignancies.