Paget disease of the nipple

Paget disease of the nipple, also known as mammary Paget disease or Paget disease of the breast, is a rare form of breast cancer that manifests as eczema-like changes of the nipple and areola. It accounts for approximately 1–3% of all breast cancers and is most commonly diagnosed in postmenopausal women, though it can occur at any age in adulthood. The condition is characterized by the presence of malignant Paget cells (large, pale-staining adenocarcinoma cells) within the epidermis of the nipple. Key symptoms include persistent redness, scaling, crusting, and flaking of the nipple skin, often accompanied by itching, burning, or tingling sensations. Patients may also experience nipple discharge (which can be bloody or serous), nipple retraction, or ulceration. Because these symptoms can mimic benign dermatological conditions such as eczema or dermatitis, diagnosis is frequently delayed. In the majority of cases (approximately 80–90%), Paget disease of the nipple is associated with an underlying breast carcinoma, either ductal carcinoma in situ (DCIS) or invasive breast cancer. A palpable breast mass may or may not be present. Diagnosis is confirmed through biopsy of the affected nipple skin, which reveals the characteristic Paget cells. Imaging studies such as mammography, ultrasound, and MRI are used to evaluate for underlying breast malignancy. Treatment depends on the extent of disease and typically involves surgical intervention, ranging from mastectomy to breast-conserving surgery (central lumpectomy with removal of the nipple-areola complex), often combined with radiation therapy. Sentinel lymph node biopsy is performed when invasive disease is suspected. Adjuvant systemic therapies, including chemotherapy, hormonal therapy, and HER2-targeted therapy, may be recommended based on the characteristics of any associated underlying breast cancer. Prognosis is generally favorable when the disease is confined to the nipple without an underlying invasive carcinoma, but outcomes depend significantly on the stage and biology of any associated breast cancer.

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