What is Paget's Disease of the Breast?

Medically Reviewed By Julie Scott, DNP, ANP-BC, AOCNP
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Paget’s disease of the breast is a rare type of cancer that affects the nipple and areola. It often only affects one breast. It mostly occurs in postmenopausal females over the age of 50.

Sex and gender exist on a spectrum. This article uses the terms “female” and/or “male” to refer to sex that was assigned at birth. 

Learn more about the difference between sex and gender here.

Early symptoms of Paget’s disease of the breast include redness or discoloration, scaling, and thickened patches of skin. Paget’s disease can look like eczema. Most females with this condition (sex assigned a birth) also have a lump in the breast.

The cause of Paget’s disease is unknown. However, various factors, such as a history of breast cancer, can increase your risk. Treatment options include surgery of the nipple, areola, or the whole breast.

This article discusses what Paget’s disease is. It also explains the symptoms, causes, diagnosis, treatments, and how to lower your risk of breast cancer.

What is Paget’s disease of the breast?

Female covering one breast
Westend61/Getty Images

Paget’s disease of the breast is the same as mammary Paget’s disease and Paget’s disease of the nipple. Paget’s disease looks like eczema and can make the nipple and surrounding area discolored, sore, and inflamed.

Paget’s disease often only affects one breast. It happens when there are cancerous cells in the surface layer of the nipple and areola. It affects 1–4% of females with breast cancer, according to the National Health Service (NHS).

It is possible to have Paget’s disease and not have another type of cancer of the breast. However, according to the American Cancer Society, 80–90% of females with Paget’s disease will also have another type of breast cancer as well.

There are two types of breast cancers linked to Paget’s disease:

  • Ductal carcinoma in situ (DCIS): With this cancer, cells that line your ducts have become cancerous. However, they have not spread to your breast tissue.
  • Invasive breast cancer: This cancer starts in your ducts and has spread to other parts of your breast tissue. 

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

Redness or discoloration around the nipple could indicate Paget's disease of the breast.

Redness or discoloration around the nipple could indicate Paget's disease of the breast.

Lily Chu, National Naval Medical Center Bethesda/Wikimedia Commons

As seen here, peeling and flaking skin on the nipple is a possible sign of Paget's disease of the breast.

As seen here, peeling and flaking skin on the nipple is a possible sign of Paget's disease of the breast.

Photography by DermNet New Zealand

Paget's disease of the breast may cause severe redness, dicoloration, or thickening of the skin around the nipple.

Paget's disease of the breast may cause severe redness, dicoloration, or thickening of the skin around the nipple.

DermNet New Zealand

Paget’s disease often only affects one breast and shares symptoms with psoriasis and eczema. Therefore, your doctor may initially misdiagnose and treat you for a different condition. Around 50% of females with Paget’s disease also experience a symptomatic lump in their breast.

Early symptoms of Paget’s disease may also improve over time and then flare up again. The first changes you may notice around your nipple or areola are:

  • redness or discoloration
  • scaling
  • crusting
  • thickened patches

Other symptoms can include:

  • a yellow discharge from your nipple
  • an itching or burning feeling
  • blood from the nipple or the areola
  • pain
  • flaky skin
  • inflammation
  • a flat or inverted nipple

Learn about the prevention and early detection of breast cancer.

What causes Paget’s disease of the breast? 

The exact cause of Paget’s disease is unknown, and further research will help doctors understand more.

Currently, are two theories on how Paget’s disease develops in the breast.

Epidermotropic theory 

This theory proposes that cancerous Paget’s cells fall away from an existing tumor in your breast. The cells then move through your milk ducts to your nipple, areola, or both.

This theory explains why some people have a lump in their breasts and changes to their nipples and areola.

Transformation theory

This theory proposes that cells in the nipple and areola can turn cancerous by themselves. This idea could explain why some people do not have a lump in their breasts.

Risk factors

Factors that increase your risk for Paget’s disease and other breast cancers include:

  • having a family history of breast cancer or breast-related conditions
  • having had breast or ovarian cancer previously
  • being over the age of 50
  • having dense breast tissue
  • drinking large amounts of alcohol
  • having exposure to radiation, certain chemicals, or UV rays
  • not eating a healthy and balanced diet
  • being overweight
  • having excess stress

How do doctors diagnose Paget’s disease of the breast?

If your doctor believes you may have Paget’s disease, they are likely to carry out the following:

  • Medical history: Your doctor will ask how long you have had your symptoms. They may also ask if you have eczema and whether you or your family have a history of breast cancer. Other questions may relate to any medications you take, such as contraceptives or hormone replacement therapy (HRT).
  • Examination: Your doctor will likely want to examine your breast. They will check the appearance of your skin and check for any lumps.
  • Tests: Your doctor may take a biopsy of your breast tissue, nipple, areola, and any discharge. They will then check your breast tissue for any cancerous cells. Other tests may include blood tests, mammograms, ultrasounds, and MRI scans.

How do you treat Paget’s disease of the breast?

Before your doctor helps you decide on your treatment plan, they will take into consideration:

  • where the cancer is in your breast
  • the stage of your cancer
  • how abnormal the cells are
  • the size of the cancer
  • other factors, such as your age and general health

Treatment for Paget’s disease may involve breast-conserving surgery, which removes the affected part of your breast. In contrast, a mastectomy is when a surgeon removes all of your breast tissue.

Your doctor may also suggest removing the lymph nodes under your arm. They will check if the cancer has spread to them or any of the muscles in your upper chest. If the cancer is in your nipple and areola, doctors may need to remove them.

Your doctor will likely recommend follow-up treatment. Treatments may include radiation therapy, chemotherapy, or HRT.

Your doctor will discuss your treatment options with you. Reconstructive surgery may be an option for your breast, nipple, and areola.

What are some tips to lower your risk of breast cancer?

The exact cause of breast cancer and Paget’s disease is unknown. However, you can take steps to reduce your chances of having breast cancer.

The Centers for Disease Control and Prevention (CDC) recommends:

  • maintaining a moderate weight
  • exercising regularly
  • limiting the amount of alcohol you consume or not drinking at all
  • eating a healthy and balanced diet, low in saturated fat
  • discussing your HRT and contraceptive options

If you have any concerns about breast cancer or Paget’s disease, talk with your doctor about preventive steps.

Learn more about breast cancer.

Summary

Paget’s disease of the breast is a rare form of cancer that affects the nipple and often the areola. Symptoms include redness, itching, and inflammation. For many females, it produces a lump in the breast. It can occur in males. However, it most commonly occurs in females over the age of 50.

Typically, treatment for Paget’s disease can be effective. Options include breast surgery, radiation therapy, and HRT. 

Eating a healthy, balanced diet and regularly exercising can reduce the chance of having Paget’s disease or breast cancer.

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Medical Reviewer: Julie Scott, DNP, ANP-BC, AOCNP
Last Review Date: 2022 Aug 30
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