What are the other Names for this Condition? (Also known as/Synonyms)

• Mammary Paget’s Disease
• Paget Disease - Breast
• Paget’s Disease of the Nipple

What is Paget’s Disease of the Breast? (Definition/Background Information)

• Paget’s Disease of the Breast is a rare form of breast cancer that starts from the nipple and extends to the areola around the nipple. The areola is the dark circle around the nipple
• The disease develops from the tumor cells, which grow in the milk ducts of the breast. Paget’s Disease of the Breast is usually associated with the underlying breast cancer
• Risk factors associated with the condition include personal history and family history of breast cancer, radiation exposure, and obesity
• The exact cause of Paget’s Disease of the Breast is not yet known. It is characterized by scaly skin of the nipple with bleeding, ulcer formation, and itching
• Paget’s Disease of the Breast is best treated with surgery. The prognosis depends on how far the underlying cancer has spread to other parts of the body

Who gets Paget’s Disease of the Breast? (Age and Sex Distribution)

• Paget’s Disease of the Breast is most commonly seen in women, who are over the age of 50 years
• The condition can affect men also, but it is very uncommon
• The average age of the affected individual is 62 years for women and 69 years for men
• Paget’s Disease of the Breast is observed worldwide; no specific race or ethnicity is affected. However, Caucasians are observed to have a higher risk of the condition

What are the Risk Factors for Paget’s Disease of the Breast? (Predisposing Factors)

Risk factors associated with Paget’s Disease of the Breast are:

• Age: The risk increases with advancing age
• Personal history of breast cancer: If the individual has already had cancer in one of the breasts, she is at a high risk of getting cancer in the other breast as well
• Family history: Individuals with a family history of breast cancer are at a higher risk
• Genetic predisposition: Defective genes in the body can make an individual susceptible to Paget’s Disease of the Breast
• Dense breast tissue: A woman with dense breast tissue has an elevated risk of Paget’s Disease of the Breast
• Radiation exposure: Radiation exposure for the treatment of some other cancer elevates the risk
• Excess weight: Obesity is a main factor for several diseases and conditions. The risk of Paget’s Disease of the Breast is increased with obesity
• Hormone replacement: Intake of estrogen after menopause increases the risk for the condition
• Race: Caucasian women are at a higher risk

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Paget’s Disease of the Breast? (Etiology)

• Paget’s Disease of the Breast arises from an underlying cancer of the breast ducts
• The cancer cells from the already existing tumor, travel to the nipple and the surrounding skin through the milk ducts and causes Paget’s Disease
• In rare cases where there is no underlying breast cancer, the nipple skin cells automatically change into cancer cells

What are the Signs and Symptoms of Paget’s Disease of the Breast?

The signs and symptoms manifested by Paget’s Disease of the Breast are:

• Rash and itchiness, formation of ulcers in the infected area
• Bleeding in the affected area
• Hardened skin on the nipple, or areola, or on both
• Scaly skin on the nipple
• Redness
• Tingling or burning sensation
• Nipple discharge
• A flattened or retracted nipple
• A lump in the breast
• Increased sensitivity
• Pain

How is Paget’s Disease of the Breast Diagnosed?

Diagnosis of the Paget’s Disease of the Breast includes:

• Breast examination: The physician examines the breast to look for the appearance of the skin in and around the nipples and for the presence of lumps, or areas of thickening
• Mammography: It is an x-ray of the breast tissue that can reveal whether the skin and nipple changes are linked with the underlying breast cancer
• Magnetic resonance imaging (MRI) scan of the breast will help detect cancer that cannot be observed through a mammography
• Breast biopsy:  A tissue sample is sent to a pathology lab for examination under a microscope. The pathologist examines the biopsy sample that can help make a definitive diagnosis. The pathologist may make additional studies to arrive at a conclusion

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Paget’s Disease of the Breast?

Complications associated with Paget’s Disease of the Breast are:

• Nipple deformity and disfigurement
• Presence of cancer in the breast and its associated complications

How is Paget’s Disease of the Breast Treated?

Paget’s Disease of the Breast is best treated using surgical procedures. The type of surgery required depends on the condition of the skin around the nipple and how advanced the cancer is.

The surgical procedure types may include the following:

• Simple mastectomy: When the underlying breast cancer has not spread to the lymph nodes, a simple mastectomy is performed in which the entire breast is removed
• Lumpectomy: Under this surgical method, only the affected portion of the breast is removed. The nipples, areola, and a cone-sized portion of the breast are removed. The focus will be on removing as little cells as possible and to keep the healthy cells intact. This surgery will require a follow-up radiation therapy

Adjuvant therapy: After the surgery, an additional therapy called adjuvant therapy with anti-cancer drugs, like chemotherapy, radiation therapy or hormone therapy, is recommended to avoid recurrence of the condition.

How can Paget’s Disease of the Breast be Prevented?

The following preventive methods could be adopted to reduce the risk of Paget’s Disease of the Breast:

• Healthy lifestyle habits
• Mild exercises
• Eating a healthy diet
• Limiting alcohol and smoking

The use of preventive medications and surgical procedures may reduce the risk of breast cancer in certain high-risk individuals.

What is the Prognosis of Paget’s Disease of the Breast? (Outcomes/Resolutions)

The prognosis for Paget’s Disease of the Breast depends on the stage of the underlying cancer and how far it has spread to other parts of the body.

Additional and Relevant Useful Information for Paget’s Disease of the Breast:

The overall prevalence and severity of Paget’s Disease are decreasing; the cause of these changes is unclear.