Breast cancer health centre

Paget's disease of the nipple

Paget’s disease of the nipple, or Paget’s disease of the breast, is a rare type of breast cancer.

Symptoms of Paget's disease of the nipple include an eczema-like itchy, red rash on the nipple and surrounding area.

Paget’s disease of the nipple usually suggests there is breast cancer in the tissue behind the nipple.

What are the possible causes of Paget’s disease of the nipple?

Scientists do not know exactly what causes Paget’s disease of the nipple, but two major theories have been suggested for how it develops. One theory proposes that cancer cells, called Paget cells, break off from a tumour inside the breast and move through the milk ducts to the surface of the nipple, resulting in Paget’s disease of the nipple. This theory is supported by the fact that more than 97% of patients with Paget’s disease also have underlying invasive breast cancer or ductal carcinoma in situ (DCIS). DCIS is a condition in which abnormal cells are present only in the lining of the milk ducts in the breast, and have not invaded surrounding tissue or spread to the lymph nodes. DCIS sometimes becomes invasive breast cancer. Invasive breast cancer is cancer that has spread outside the duct into the breast tissue, and possibly into the lymph nodes under the arm or into other parts of the body.

The other theory suggests that skin cells of the nipple spontaneously become Paget cells. This theory is supported by the rare cases of Paget’s disease in which there is no underlying breast cancer, and the cases in which the underlying breast cancer is found to be a separate tumour from the Paget’s disease.

What are the symptoms of Paget’s disease of the nipple?

Symptoms of early Paget’s disease of the nipple include redness and mild scaling and flaking of the nipple skin that is very similar in appearance to eczema. Early symptoms may cause only mild irritation and may not be enough to prompt a visit to the doctor. Improvement in the skin can occur spontaneously, but this should not be taken as a sign that the disease has disappeared. More advanced disease may show more severe destruction of the skin. At this stage the symptoms may include tingling, itching, increased sensitivity, burning and pain. There may also be discharge from the nipple, and the nipple can appear flattened against the breast.

In approximately half of patients with Paget’s disease of the nipple, a lump or mass in the breast can be felt during physical examination. In most cases Paget’s disease of the nipple is initially confined to the nipple, later spreading to the areola or other regions of the breast. The areola is the circular area of darker skin that surrounds the nipple. Paget’s disease of the nipple can also be found only on the areola, where it may resemble eczema. Although rare Paget’s disease of the nipple can occur in both breasts.