The Perianal Mirage: Case of Primary Perianal Paget’s Disease

Background: Paget’s disease is rare and arises from the apocrine glands of skin. Perianal Paget’s disease is very rare form of extramammary Paget’s disease. Method and Results: Patient presented with complaints of perianal itching which on examination had patchy depigmented nodular skin in perianal region. Biopsy showed Paget’s cells, and immunohistochemical staining was positive for CK7, CEA and GCDFP-15, confirming the diagnosis of primary perianal Paget’s disease. Further, patient was staged and was planned for surgery and underwent wide local excision of perianal skin preserving internal and external anal sphincters. Reconstruction of the defect was completed with a local flap. Conclusions: Surgery remains most effective and only curative option for perianal Paget’s disease. As it is a rare disease, a high degree of suspicion is required for early diagnosis and treatment.