Paget’s Disease of the Breast - An Overview

What Is Paget’s Disease of the Breast?

Paget’s disease of the breast is a rare type of cancer. It develops in the nipple's skin and sometimes in the areola (dark skin surrounding the nipple).

Breast eczema-like changes may occur on the skin of the nipple and the area of darker skin surrounding it (the areola). Mostly, these eczemas can be mistaken for an ordinary rash.

This condition may be confined to nipples (ductal carcinoma in situ), or the condition may be accompanied by breast cancer that has invaded the milk ducts of the same breast.

This type is also known as Paget’s disease of the breast. A multidisciplinary approach, a team of doctors, and a planned treatment approach help manage the condition.

Who Gets Paget’s Disease of the Breast?

Paget’s disease of the breast occurs among individuals over 50 years old. It is more common among females; rare cases occur in males. The average age of diagnosis is 57 years, but it may occur at a younger or older age. Paget’s disease may be observed in less than four percent of breast cancers.

What Causes Paget’s Disease of the Breast?

In Paget's disease of the breast, the cancer cells form in the milk ducts and then extend and spread to the skin of the nipple and areola. These cells proliferate among the normal skin cells, allowing fluid to seep out and creating cracks or a scaly, crusty patch on the nipple.

Under a microscope, the cells appear aberrant with large nuclei and pale cytoplasm, frequently accompanied by skin changes and inflammation. Paget's disease may result from underlying breast cancer (DCIS) or from unique cells in the nipple known as Toker cells.

Scientists do not know exactly what causes it, but they think it might be related to things like long-term exposure to chemicals, changes in your DNA, or unhealthy habits like smoking and drinking too much.

What Are the Symptoms of Paget’s Disease of the Breast?

The symptoms of Paget’s disease of the breast are most often noticed in the nipple and/or areola. Generally, only one breast is affected. In rare conditions, both breasts are affected. Symptoms usually appear and then go, but eventually they worsen.

The symptoms of Paget's disease of the breast include:

• Nipple itching is present, which may progress to a burning sensation.

• The skin on the nipple or areola appears dry, scaly, and flaky.

• In this case, the rash appears red, raised, crusted, raw, or bleeding. The important thing to notice is that, in the beginning, a breast cancer rash appears like a bruise that does not go away. These are caused by the blockage of lymph vessels in the breast.

• It is not easy to deal with this condition because your skin may be crusty, oozing, and itchy or bumpy, which is a lot like eczema. If a common rash or eczema treatment isn't working, that is a key sign to see your doctor.

• Nipple discharge may contain blood or pus.

• Nipples may be flat or inverted.

• A lump may be present in the breast, and that may be palpable.

How Is Paget’s Disease of the Breast Diagnosed?

Doctors typically treat Paget's disease as simple eczema at first because it frequently appears as a benign rash. Your doctor will consider additional diagnostic testing if your symptoms do not go away.

• Immunohistochemistry (IHC).

• Mammogram.

• Blood tests.

• Ultrasonography (USG).

• Fine needle aspiration cytology (FNAC).

• Computed tomography (CT).

• Magnetic resonance imaging (MRI).

How Is Paget’s Disease of the Breast Treated?

The treatment of Paget’s disease of the breast involves surgery. Sometimes, it may be accompanied by adjuvant therapies. These adjuvant therapies help prevent recurrence of the condition. The first phase of treatment includes steroid therapy to relieve symptoms.

Other treatments include the following procedures.

• Surgery: Mastectomy or surgical removal of the lump in the breast, is done.

• Chemotherapy: The doctor may prescribe drugs like CHOP (Cyclophosphamide, Doxorubicin hydrochloride (Hydroxydaunorubicin), Vincristine sulfate (Oncovin), and Prednisone) for six cycles: Cyclophosphamide 750 milligrams per square meter (mg/m²) intravenously on the first day, Doxorubicin 50 mg/m² on the first day, Vincristine 1.4 mg/m² on the first day, and Prednisone 100 mg orally daily from the first to the fifth day. The drug Rituximab is effective against CD20+ B-cell lymphomas.

• Radiotherapy: If the lump persists, radiation therapy is done after the chemotherapy session.

• Hormone Therapy: Hormones are chemical messengers that regulate many functions in the body. Hormones play a role in cancer growth. Hormone therapy cuts off the hormones that help in the growth of cancer.

• Immunotherapy: It improves the immune system's ability to fight cancer. This therapy boosts the immune system and helps in killing cancerous cells.

Paget’s disease is also observed during pregnancy and postpartum under the influence of various hormones. About 80% of Paget’s breast lymphomas are B-cell lymphomas that are CD20-positive.

What Are the Risk Factors for Paget’s Disease of the Breast?

Factors that increase the risk of breast cancer are also responsible for the development of Paget’s disease.

These include:

• Aging.

• Personal history of breast cancer in one breast or both, such as atypical hyperplasia (an abnormal increase in cell number of the breast).

• Family history of breast cancer.

• Obesity.

• Radiation exposure due to some treatments at a younger age.

• Certain gene mutations, such as Breast Cancer gene 1 (BRCA1) and Breast Cancer gene 2 (BRCA2), can also increase the risk of breast cancer.

• Early periods, before age 12.

• Menopause starts at an older age.

• Giving birth or having a first baby after the age of 30.

• Receiving hormonal therapy postmenopause.

• Women who have never been pregnant are also at risk of developing breast cancer.

What Are the Preventatives for Paget’s Disease of the Breast?

Paget’s disease of the breast can be prevented by making changes in daily life, such as

• Consult the doctor to get screened for breast cancer, and also ask about the pros and cons of screening tests.

• Self-examination of the breast should also be done to look for lumps or other changes, including rashes, and consult with the doctor immediately.

• You can exercise regularly for at least 30 minutes a day, which can also be beneficial.

• Alcohol consumption should also be in moderation, that is, two drinks for men and one drink for women in one day. One drink means a 5-ounce glass of wine.

• Maintaining a healthy weight and opting for healthy weight management strategies can also be effective in reducing the risk of breast cancer.

• You can also speak to your doctor and limit hormonal therapies postmenopause.

• A healthy diet, such as a Mediterranean diet rich in nuts and extra-virgin olive oil, also reduces the risk of breast cancer.

Conclusion:

Paget’s disease of the breast is the rarest form of malignancy, with signs like breast cancer rash, nipple eczema, etc. The condition can be cured based on the cancer stage and early detection. In the case of stage 0, that is, if the cancer is confined to the nipples only and has not spread beyond the milk ducts, Paget's disease shows an excellent prognosis. However, if the cancer starts spreading, it becomes deadly and challenging to cure. Therefore, early screening for the disease can lead to better outcomes. This condition can also develop in men, but very rarely. Connecting with an oncology specialist at iCliniq experienced in rare breast cancers can offer clarity and confidence.

Key Takeaways:

• Rarely affecting the nipple and areola, Paget's disease of the breast frequently resembles rashes or eczema.

• Early detection is crucial because it may be limited to the nipple (DCIS) or linked to invasive breast cancer.

• Itching, burning, crusting, nipple discharge, or lumps typically in one breast are major symptoms.

• For diagnosis, doctors generally recommend imaging tests, a biopsy, and immunohistochemistry to confirm the presence of Paget cells.

• Treatment, such as surgery and early specialized care, enhances outcomes. Immunotherapy, hormone therapy, chemotherapy, and radiation are also advised when needed.