What to know about Paget's disease of the breast

Share on Pinterest

Paget’s disease of the breast causes skin symptoms on the breasts or nipples because of underlying breast cancer. In 80–90% of cases, the underlying cancer is either DCIS or infiltrating ductal carcinoma, also known as invasive breast cancer.

Paget’s disease of the breast may resemble other common skin issues, such as wounds, breastfeeding-related injuries, or eczema.

The cancer begins in the epidermis of the nipple or areola. Researchers think this occurs because cancerous cells in the breast skin move to the outer portion of the breast through milk ducts.

Paget’s disease of the breast is a rare type of breast cancer that primarily affects females. However, rare cases have occurred in males.

This disease can affect people aged 26–88 years, but it occurs more commonly in postmenopausal females in their 50s.

This condition represents about 1–4% of all breast cancer cases. However, experts do not know the exact incidence and frequency of Paget’s disease of the breast in the general population.

Paget’s disease of the breast causes changes in the texture or appearance of the skin of the breasts or nipples. In most cases, the changes occur on the nipple or areola.

Some signs to look for include:

• discoloration on the nipples or areola
• crusting or discharge from the nipple
• swelling or changes in the texture of the skin
• marks that look like wounds or injuries on the nipple

The skin changes may hurt or itch, and the changes in the appearance of the breast tissue range from subtle to dramatic.

Early stage

In the early stages of Paget’s disease of the breast, the symptoms may be subtle. A person might mistakenly believe that they have eczema, skin irritation, or a breastfeeding-related infection.

One of the most common early signs is an itchy growth that disappears or gets better but then comes back. It may have tiny blisters. Some other signs to look for include:

• any patch of dry or bumpy skin that does not go away
• any apparent injury that either does not heal or heals but comes back
• eczema on the breast or nipple that does not heal
• any change in the nipple or the skin of the breast
• changes in the way the nipple feels

When Paget’s disease of the breast presents without a palpable mass — meaning one that a person can feel through touch — it is usually not an aggressive cancer. The aggressiveness of the cancer, though, ultimately depends on the underlying cause of Paget’s.

Death rates from DCIS are very low among those who receive treatment. However, DCIS can increase the risk of eventually dying from another type of breast cancer.

More advanced cancers, especially invasive ductal carcinoma, have a higher mortality rate. Overall, the 10-year survival rate with Paget’s disease of the breast is 82% if a person cannot feel a mass and a doctor removes the cancer. This reduces to 22% if the mass is palpable.

The factor that plays the most significant role in determining the outcome is early diagnosis. By the time there is a large mass or the cancer has spread to lymph nodes, the disease is more aggressive and more likely to be fatal.

The symptoms of Paget’s disease vary depending on the stage of development. However, a person cannot diagnose the stage of cancer based on the symptoms alone.

The most common symptoms, which usually only occur in one breast or nipple, 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
• a mass in the nipple
• ulceration, in some cases

About 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.

In the early stages of Paget’s disease of the breast, a person might notice:

• nipple pain or itching
• changes in the color or shape of the nipple
• changes to breast skin, such as eczema-like marks or a wound that does not go away

In the later stages of the disease, the following symptoms are more common:

• a lump or mass in the breast
• swelling or pain in the lymph nodes
• more extensive and noticeable skin symptoms, such as significant areas of redness or discoloration

Doctors may not immediately diagnose Paget’s disease of the breast because of its similarity 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 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 though 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 that 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, either 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 for Paget’s disease 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 close relative with breast cancer
• having denser breast tissue
• having a genetic mutation associated with breast cancer, such as BRCA1 or BRCA2
• hormone replacement therapy (HRT)
• excessive alcohol consumption

Learn about how quickly breast cancer can spread.

Doctors may use a variety of tests to detect the presence of breast cancer and treat it accordingly:

Diagnosis

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:

• a mammogram
• an ultrasound or MRI scan
• a biopsy

If breast cancer is present, the characteristics and stage of the condition will determine the treatment options. These may include surgery, targeted therapy, and chemotherapy.

Surgery

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

A doctor may recommend modified radical mastectomy. During this procedure, a surgeon will remove the breast, the lining over the chest muscles, and some of the lymph nodes under the arm.

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 only affects the nipple and the surrounding area, the doctor may suggest surgery to conserve the breast.

This type of surgery, known as lumpectomy, involves removing the nipple, the 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.

Learn about nipple reconstruction surgery.

Hormone treatment, targeted therapy, and chemotherapy

Depending on the type of cancer, a doctor may recommend drug therapy with either hormones or medications that target genes specific to cancerous cells.

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 there is a tumor in the breast
• the type of cancer, if a tumor is present
• the stage of the cancer, if the cancer is invasive

Lymph node swelling can also affect a person’s outlook.

Statistics on survival

Statistics show high rates of survival when there is not a palpable tumor. Following excision, 92% of people survive at least 5 years, and 82% survive at least 10 years. However, if there is a palpable breast mass, only 38% of people survive 5 years.

According to the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) Program, the 5-year relative survival for all females in the U.S. who received a diagnosis of Paget’s disease of the breast between 1988 and 2001 was 82.6%. This is slightly lower than the rate of 87.1% among females with a diagnosis of any type of breast cancer.

However, for people with both Paget’s disease of the breast and invasive breast cancer, the 5-year relative survival falls in the later stages of cancer. For example, in stage 4 — where the cancer has spread to other parts of the body — the 5-year relative survival rate is 14.3%.

For this reason, people should seek advice as soon as possible if they notice any changes in a breast or nipple.

Paget’s disease of the breast is not usually an aggressive breast cancer. However, it can spread if a person does not seek prompt treatment.

As it can mimic other skin conditions, a person may delay care, which could allow the cancer to spread. People who develop any marks or skin issues on the breasts should see a doctor, even if they think a serious underlying medical condition is unlikely.

With early diagnosis and treatment, Paget’s disease of the breast is typically curable.