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

Symptoms differ from those of other types of breast cancer. Paget’s cells can be found on the surface of the skin of your nipple and areola.

The exact cause of this type of breast cancer isn’t clear. One common theory is that cells from a tumor migrate through the milk ducts to reach the nipple and areola. Some people who develop Paget’s disease of the breast don’t have breast tumors, however.

Keep reading and find out how to identify Paget’s disease of the breast and why diagnosis is often delayed.

For many types of breast cancer, the first symptom is a lump in the breast. Learn what a breast cancer lump feels like.

In Paget’s disease of the breast, there’s another sign you’re likely to notice first. It involves the nipple and areola of your breast, where you might have:

  • redness
  • flaking, crusting, or scaling
  • a tingling or itching sensation

It would be easy to mistake these symptoms as eczema, dermatitis, or some other skin condition. Paget’s disease of the breast is usually found on only one breast, and it won’t respond to topical treatments.

Here are some other clues that signal something more than a skin condition:

  • thickening skin on or near your nipple
  • flattening of your nipple
  • yellowish or bloody discharge from your nipple
  • increasing breast sensitivity, soreness, and pain

If you have any of these signs and symptoms, see your doctor as soon as possible.

The exact prevalence and incidence of Paget’s disease of the breast in the general population isn’t known. We do know that it represents a small portion of all breast cancers, and it’s seen in between 1 and 4 percent of breast cancer cases.

There are some factors that may increase your risk of developing breast cancer:

  • You’re a woman over age 50.
  • You have a history of breast cancer or other breast abnormalities.
  • You have close relatives who have had breast or ovarian cancer.
  • You carry mutations of the BRCA1 or BRCA2 genes.
  • You have dense breast tissue.
  • You’ve had previous radiation treatment to your chest.
  • You’re overweight, especially after menopause.
  • You take hormone replacement therapy.
  • You regularly drink a lot of alcohol.

Risk factors specific to Paget’s disease of the breast aren’t clear.

Diagnosis begins with a physical examination. Your doctor will observe the appearance of your breasts, especially around the nipples, and check for lumps or unusual thickening.

It’s likely that your doctor will also order a diagnostic mammogram to look for signs of breast cancer. If there’s anything unusual or unclear, this may be followed by an ultrasound or MRI scan. These detailed images can help pinpoint areas of concern. See examples of mammogram images.

The only way to confirm breast cancer is with a biopsy. This is a procedure in which a needle is used to obtain a small sample of breast tissue from a nipple, areola, or tumor. The sample is then sent to a pathologist who will use a microscope to look for cancer cells.

The large, round appearance of Paget’s cells can confirm the disease. The biopsy can also provide other important details, such as:

Your oncologist will come up with a treatment plan that takes many factors into account, such as:

  • the size and grade of breast tumor, if any
  • if the cancer has spread (stage)
  • HR and HER2 status
  • if you’ve been treated for cancer in the past
  • your age and general state of health

The type of surgery you have depends on the number and location of tumors, and the relative size of your breast. In breast-conserving surgery, the nipple and areola are removed. This may be a viable option if you don’t have breast tumors.

If you do have breast tumors, removing the whole breast (mastectomy) may be recommended. In this case, your surgeon might also want to biopsy your sentinel lymph nodes to see if the cancer has spread. If so, more extensive lymph node surgery may be necessary.

Surgery might be followed by radiation therapy to target any cancer cells that were missed. Powerful chemotherapy drugs may be used to seek and destroy cancer cells, no matter where they’ve traveled in your body.

If your tumor is HR-positive or positive for overexpression of the HER2 protein, you may be able to take advantage of additional targeted treatments.

Breast cancer treatment usually consists of a combination of therapies.

One potential complication of this type of breast cancer is that symptoms can be easily dismissed or misdiagnosed, delaying treatment. Cancer is easier to treat in its early stages.

Surgery, radiation, chemotherapy, and hormone treatments can lead to a variety of temporary complications such as pain, fatigue, and skin irritation.

Breast cancer treatment can increase your long-term risk of a variety of conditions, such as:

  • lymphedema, due to removal of lymph nodes
  • increased risk of other cancers, as a result of chemotherapy or radiation therapy
  • early menopause or infertility, caused by chemotherapy or hormone treatments

Your outlook depends on many factors, such as:

  • whether you have a tumor and if it’s invasive or noninvasive
  • tumor grade and stage at diagnosis
  • HR and HER2 status
  • other health conditions
  • how well you respond to therapy

If you have Paget’s disease of the breast but no tumors or lymph node involvement, your prognosis is excellent. The survival rate may be reduced if the cancer is invasive and has spread to the lymph nodes.

With your complete medical profile in mind, your doctor can give you an idea of your personal prognosis.