Abstract
INTRODUCTION AND IMPORTANCE: Paget's disease of the nipple is a rare form of ductal carcinoma in situ. It is often associated with an underlying breast cancer or, more rarely, may be isolated with no associated breast neoplasm. PRESENTATION OF CASE: We report the case of a patient with extensive Paget's disease of the nipple without associated breast lesions, treated by mastectomy with coverage by a dorsalis major flap. CLINICAL DISCUSSION: The association of Paget's disease of the nipple with breast cancer is the most frequent situation, requiring a complete radiological work-up of the breast. The diagnosis is confirmed by anatomopathological examination combined with immunohistochemistry, the treatment of Paget's disease of the nipple remains controversial, with conservative or radical treatment depending on the extent of the disease and the presence or absence of associated breast cancer. CONCLUSION: Paget's disease of the nipple is a rare form of breast cancer. Treatment relies mainly on surgery, which can be either conservative or radical.