Perianal Paget’s Disease: A Report of a Rare Case

This case report discusses a 56-year-old Saudi female patient diagnosed with extramammary Paget's disease (EMPD) affecting the perianal region. Initially presenting with a one-year history of perianal itching and erythematous plaques, she underwent multiple evaluations, including a punch biopsy that revealed atypical epidermal infiltrates suggestive of EMPD or squamous cell carcinoma in situ. Comprehensive imaging and additional biopsies confirmed the diagnosis and assessed the extent of the disease. The patient underwent laparoscopic sigmoid colostomy and a wide local excision, followed by reconstructive procedures involving multidisciplinary surgical teams. Postoperative pathology confirmed perianal Paget's disease (PPD) with positive resection margins at the proximal vaginal area. The patient was discharged in stable condition and continues to receive follow-up care, highlighting the importance of multidisciplinary management in complex cases of perianal premalignancies and malignancies. Notably, to the best of our knowledge, no other case report has addressed a lesion of this size. Additionally, the initially uncertain location of the primary cancer lesion further distinguishes our case. Further studies are warranted to monitor for disease recurrence and evaluate the need for additional interventions.