ABSTRACT

Paget's disease of the breast is a rare disorder that affects the nipple-areola complex and is usually associated with underlying ductal carcinoma in situ and/or invasive ductal cancer. It usually presents with eczema-like changes in the nipple and areola, with a thickened, erythematous, sometimes pigmented, crusted lesion with irregular borders, and is almost always unilateral. Serous and bloody discharge may occur, and ulceration and destruction of the nipple-areola complex may result. The diagnosis is usually by triple assessment, and radiological findings are important to determine further management. The treatment of Paget's disease is still controversial, with mastectomy with or without axillary lymph node dissection being regarded as the standard therapy in the past. Nowadays, data suggest that local control may be achieved with breast conservation surgery with negative margins followed by whole-breast radiation therapy.