Paget's disease of the breast or nipple refers to breast changes that can indicate that cancer cells are forming in or around the nipple. A rash on or around the nipple may mean that changes are occurring beneath the skin.

Usually, Paget's disease occurs alongside another type of cancer — usually ductal carcinoma in situ (DCIS) or invasive breast cancer — elsewhere in the breast.

Paget's disease is more common in women, but it can also affect men. It usually appears after the age of 50 years. The average age at diagnosis is 62 for women and 69 for men, but it can happen at any age. Between 1% and 4% of breast cancer cases involve Paget's disease.

The symptoms can resemble those of a skin condition, such as eczema or psoriasis. This sometimes delays diagnosis, increasing the risk of a life-threatening condition.

Learn more here about breast cancer.

woman with breast discomfort that may be pagets diseaseShare on Pinterest
Paget's disease of the breast may cause tingling and itching.

The symptoms of Paget's disease vary depending on the stage of development. They usually only occur in one breast.

The main symptoms include:

  • itching, tingling, or burning around the nipple and areola
  • redness or pigmentation
  • flaking, thickening, or scaling of the skin around the nipple that does not heal over time
  • nipple discharge, which may be yellow or contain blood
  • flattened or inverted nipples
  • a crusted lesion with irregular edges
  • ulceration, in some cases

These usually affect only one nipple.

Around half of all people with Paget's disease of the breast also have a breast lump behind the nipple. In 90% of these cases, invasive breast cancer is present.

Doctors often find Paget's disease challenging to diagnose due to its resemblance to dermatitis and eczema. The latter, unlike Paget's disease, usually affects the areola first and then the nipple.

A person may not visit the doctor because they mistake the condition for contact dermatitis or eczema. Anyone who notices a lump or skin irritation that does not heal within 1 month should seek the opinion of a specialist.

A person may also experience crusty, oozing, or hardened skin resembling eczema on the nipple, areola, or both. The skin changes may fluctuate early on, making it appear as if the skin is healing.

A burning sensation may start to occur as the disease progresses. At this point, serious destruction of the skin often prompts people to visit a doctor.

Doctors do not know the exact cause of Paget's disease of the breast. One theory is that a person already has cancer of the breast, and some cancer cells migrate through the milk ducts to the surface of the nipple.

Another possible explanation is that the skin cells of the nipple spontaneously transform into cancer cells. In a few cases of Paget's disease, there is no underlying breast cancer, or if a tumor is present, it has no link to the disease in the nipple.

Risk factors

The risk factors are similar to those for other types of breast cancer.

They include:

  • age, as the risk increases over time
  • a previous personal history of breast cancer or breast abnormalities
  • having a relative with breast cancer
  • having denser breast tissue
  • excess weight or obesity
  • hormone replacement therapy (HRT)
  • excessive alcohol consumption

How quickly breast cancer spreads will depend on whether is it invasive or not. Learn more here.

A doctor will examine any unusual areas of the breast, especially the skin on and around the nipples and feel for any lumps or areas of thickening.

To confirm the diagnosis, a doctor may recommend:

Treatment will depend on the findings. If breast cancer is present, the doctor will recommend treatment depending on the characteristics and stage of the condition.

Surgery

A person may need surgery, and this may be the only option if the changes affect a large area of the breast.

Invasive cancer or ductal carcinoma in situ (DCIS): In these cases, the doctor may recommend a modified radical mastectomy. A surgeon will remove the breast, the lining over the chest muscles, and some of the lymph nodes under the arm.

Non-invasive breast cancer: If the underlying breast cancer is not invasive, the surgeon may perform a simple mastectomy to remove only the breast and the lining over the chest muscles.

After surgery, breast reconstruction can restore the appearance of the breast.

If cancer is present, but it affects only the nipple and the surrounding area, the doctor may suggest surgery to conserve the breast, or lumpectomy, followed by radiation therapy.

This type of surgery involves removing the nipple, areola, and the entire portion of the breast that contains the cancerous cells. Most people will then have radiation therapy to help prevent the cancer from returning.

What is nipple reconstruction surgery? Learn more here.

Hormone treatment, targeted therapy, and chemotherapy

Depending on the type of cancer, a doctor may recommend drug therapy, either with hormones or with medications that affect how a specific gene works.

These treatments can reduce the risk of the cancer spreading.

Chemotherapy may help prevent the cancer from returning after treatment, especially if:

  • the tumor was large
  • the cells had spread to the lymph nodes
  • the cells are of an aggressive type

After treatment finishes, it is essential to attend all follow-up appointments and to continue to monitor for changes.

The outlook will depend on various factors, including:

  • whether or not there is a tumor in the breast
  • if cancer tumor is present, which type of cancer it is
  • if the cancer is invasive, the stage of the cancer

Statistics show that people who receive a diagnosis of Paget's disease and invasive breast cancer in the same breast have a 95.8% chance of surviving another 5 years or more.

If the person receives a diagnosis at the later stages, when cancer has already spread to distant parts of the body, the chance of surviving another 5 years is 14.3%.

For this reason, people should seek advice as soon as possible if breast changes occur.