Paget’s disease is a rare form of breast cancer that appears around and in the nipple and typically indicates that there is breast cancer under the skin. Menopausal women are most often affected, but younger woman as young as 20 may experience this condition. Paget’s disease of the nipple causes are not fully known.
Signs and Symptoms
During the early stages of this disease, symptoms may include redness, mild skin irritation and flaky and scaly skin.
As this condition progresses into the advanced stages, other signs and symptoms may occur. These include:
- Nipple skin tingling
- Nipple has very sensitive skin
- Bloody discharge or ooze from the nipple
- Areola skin has a scaly rash
- Itchiness that does not get better with creams
- Painful or burning nipple skin
- Nipple retraction
- The affected skin has a breast lump beneath it
Causes and Risk Factors
The average age of diagnosis for this condition is 62 years of age for women and 69 years of age for men. Paget’s disease of the nipple’s exact causes are unknown, but there are two theories as to how this condition occurs. One theory believes that Paget cells, a type of cancer cell, break off of a tumor within the breast and then travel through the milk ducts to the nipple’s surface. This theory is supported due to 97 percent of patients that have this condition also have underlying ductal cell carcinoma in situ or invasive breast cancer.
The second theory believes that Paget cells form when nipple skin cells spontaneously become them. This theory is supported since there is no underlying breast cancer in rare cases of Paget’s disease. And, when underlying breast cancer is present, the associated tumor is separate from Paget’s disease.
Risk factors won’t necessarily cause this condition, but it can increase a person’s risk of developing it. Some of the most common risk factors include:
- As a person gets older, there risk of developing this condition increases
- A personal medical history of breast abnormalities increases a person’s risk, specifically atypical hyperplasia or lobular carcinoma in situ
- A genetic predisposition may play a role, especially being BRCA1 or BRCA 2 positive
- Being exposed to radiation can increase a person’s risk
- Taking hormone replacement therapy can increase the risk
- A personal medical history of breast cancer can increase the risk
- A family history of ovarian cancer, breast cancer, or both can increase the risk
- Having dense breast tissue can increase the risk
- Being overweight or obese can increase the risk, especially if the extra weight was gained as an adult and after menopause
- White women are at the highest risk
National Cancer Institute. (2010). Paget Disease of the Nipple. Retrieved on November 21, 2010 from the National Cancer Institute: https://www.cancer.gov/cancertopics/factsheet/sites-types/paget-breast
MayoClinic.com. (2010). Paget’s Disease of the Breast. Retrieved on November 21, 2010 from MayoClinic.com: https://www.mayoclinic.com/health/pagets-disease-of-the-breast/DS00771