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This rare form of breast cancer is easiest to treat when it's caught early.

Paget's disease of the breast is among the rarest types of breast cancer, occurring with only 1% to 4% of all cases, says the National Cancer Institute (NCI). It often appears as skin inflammation or scaliness on one nipple.

Unfortunately, this unusual symptom of breast cancer may be dismissed as skin irritation. This can cause a delay in diagnosis and treatment, giving the cancer a chance to spread.

The disease gets its name from the doctor who discovered it, Sir James Paget. Dr. Paget also discovered diseases of the bones, eyelids and other body parts. All of the diseases are named after Dr. Paget, but beyond that they have nothing in common.

Abnormal growth

Like all cancers, Paget's disease of the breast develops when normal cells become abnormal and then divide and grow uncontrollably.

In Paget's disease this process starts in the breast ducts, the tiny tubes that carry milk to the nipple during breastfeeding.

Symptoms to look for

According to the NCI, symptoms of Paget's disease may include:

• Redness, scaling and flaking of the nipple.
• Tingling or itching around a nipple.
• A lump or mass in the breast.

Paget's disease usually occurs in only one breast.

Checking it out

If you have any of the symptoms of Paget's disease of the breast, see your doctor. A mammogram (x-ray of the breast) or biopsy (tissue sample) or other testing may be recommended to check for signs of cancer in the nipple or breast.

If there's cancer in only the nipple, surgery to remove the nipple may get rid of the cancer. If the cancer has spread beyond the nipple, other treatment will probably be needed. Treatments may include surgery, chemotherapy or radiation therapy. 

The best treatment for each case depends on several factors, including the location of the tumor, how far the cancer has spread and the person's overall health.

Reviewed 2/26/2025