Cancer Breast Cancer More Subtypes An Overview of Paget's Disease of the Breast A rare type of breast cancer affecting the nipple or areola By Pam Stephan Pam Stephan is a breast cancer survivor. Learn about our editorial process Pam Stephan Medically reviewed by Medically reviewed by Doru Paul, MD on August 19, 2019 Doru Paul, MD, is triple board-certified in medical oncology, hematology, and internal medicine. He is an associate professor of clinical medicine at Weill Cornell Medical College and attending physician in the Department of Hematology Oncology at the New York Presbyterian Weill Cornell Medical Center. Learn about our Medical Review Board Doru Paul, MD on August 19, 2019 Print Table of Contents View All Table of Contents Causes Diagnosis Treatment Paget’s disease of the breast (also known as Paget’s disease of the nipple) is a rare type of breast cancer in which cancer cells are found on the surface of the nipple and the dark circle surrounding it (the areola). Paget’s disease of the breast itself does not cause breast lumps or tumors. However, the majority of people with Paget's have either invasive breast cancer or ductal carcinoma in situ (DCIS), and related breast tumors are almost always found in the same breast that has Paget's disease cells. Paget’s accounts for 5% of all breast cancers. It most commonly affects people in their 50s, especially menopausal women. That said, the disease may also appear in adolescents and the elderly. Male breast cancers only account for 0.5% of breast cancers, and Paget’s in men is even rarer. Paget's disease was named after Sir James Paget, a British doctor from the 1800s who published articles about the link between changes to the nipple and underlying breast cancer. Verywell / Gary Ferster Symptoms Paget’s disease causes the skin on the nipple and the area around it to be red, sore, flaky, and scaly. Early on, these symptoms will come and go. Symptoms will worsen with time and may include: Itching or a burning sensation in the nipple and/or areolaPain and sensitivityFlattening of the nippleYellow or bloody discharge from the nippleA lump in the affected area Symptoms of Paget’s disease are sometimes mistaken for symptoms of skin conditions, including eczema or dermatitis. Paget's is usually only found in one breast and does not respond to topical medications. If you have any of the signs or symptoms noted above, see you doctor as soon as possible. Causes Doctors don't know exactly what causes Paget’s disease of the breast. Most researchers think the disease is the result of an underlying breast issue; more than 97% of people with Paget's also have DCIS or invasive cancer somewhere else in the breast, according to the nonprofit Breastcancer.org. Cancer cells may travel from the tumor up through the milk ducts, where they enter the nipple and areola. In a few cases of Paget’s disease, there is no underlying breast cancer, or if a tumor is present, it is unrelated to the disease in the nipple. In these cases, nipple skin cells may spontaneously change into cancer cells. Risk factors that increase the risk of any type of breast cancer are also associated with Paget’s disease. These include: Increasing ageRace: White women have a higher risk of breast cancer than black and Hispanic women.Family history (mother, sister, or daughter) of breast or ovarian cancerPersonal history of breast cancer in one breast, lobular carcinoma in situ (LCIS), atypical hyperplasia, or certain benign breast conditionsInherited gene mutation (e.g., BRCA1 or BRCA2)Dense breast tissuePrevious radiation exposure to the chestUse of hormone replacement therapy after menopauseBeing overweight, especially after menopauseExcessive alcohol consumption What Is Known About the Causes of Breast Cancer Diagnosis If you are experiencing any of the above signs and symptoms, see a health professional. Diagnosing Paget's disease will involve the following steps: Clinical breast exam: Up to 50% of people with Paget's disease of the nipple have a breast lump or an area of thickening that is felt upon clinical exam. Mammography: In Paget’s disease of the breast, a mammogram may reveal nipple and skin changes that are linked to underlying breast cancer. If a mammography of both breasts doesn’t show anything unusual, your doctor may follow up with magnetic resonance imaging (MRI).Breast biopsy: During a breast biopsy, a small sample of tissue is taken from the skin of the nipple for examination under a microscope (a sample will also be taken from the lump, if present). If there is nipple discharge, it will be collected and examined as well.Sentinel lymph node biopsy: In this procedure, a surgeon locates and removes sentinel nodes—the first place breast cancer cells go. If a sentinel node is examined and found to be negative, it is unlikely that cancer will be found in the remaining nodes. If cancer cells are detected in any of the biopsy samples, your doctor will refer you to a surgeon to discuss treatment. Treatment Surgery is the primary treatment option for Paget's disease of the breast. There are two options: Lumpectomy: This breast-conserving surgery removes only the diseased portion of the breast. With Paget’s disease, the surgeon removes the nipple and areola, along with a cone-shape part of the breast. Care is taken to spare as much tissue as possible while ensuring that the cancer cells are removed.Mastectomy: If the underlying breast cancer is invasive and removing the entire breast makes more sense, a mastectomy will be done instead. If lymph nodes are affected, these will be removed with a sentinel lymph node biopsy. After surgery, your doctor may recommend additional treatment with anti-cancer drugs (chemotherapy), radiation, or hormone therapy to prevent a breast cancer recurrence. For example, if a lumpectomy surgery is used to treat Paget’s disease of the breast, it is usually followed with radiation therapy to kill any cancer cells that may be in the remaining breast tissue. Many women will have nipple reconstruction after their lumpectomy and once radiation is completed. The specific treatment that's recommended will depend on the extent of the cancer and the nature of the tumor (for example, if the tumor has estrogen or progesterone receptor). How Breast Cancer Is Treated A Word From Verywell Since Paget's disease is not common, you've likely heard less about it than more common types of breast cancer. In addition, it can be difficult to find a Paget's support group in many communities due to the limited number of people with the disease. Fortunately, online communities give you the ability to connect with people around the world who share your diagnosis. Consider reaching out for support from others facing your same challenges. Support Groups Can Give Cancer Patients Much Needed Help Was this page helpful? Thanks for your feedback! Get honest information, the latest research, and support for you or a loved one with breast cancer right to your inbox. Sign Up You're in! Thank you, {{form.email}}, for signing up. There was an error. Please try again. What are your concerns? Other Inaccurate Hard to Understand Submit Article Sources Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy. Breastcancer.org. Paget's Disease of the Nipple. Calla VR & Deshmane V. Challenges in Diagnosis and Management of Paget’s Disease of the Breast—a Retrospective Study. Indian J Surg (2015) 77(Suppl 3): 1083. doi:10.1007/s12262-014-1167-6 Daly B & Olopade OI. Race, Ethnicity, and the Diagnosis of Breast Cancer. JAMA. 2015 Jan 13; 313(2): 141–142. doi:10.1001/jama.2014.17323 Karakas C. Paget's disease of the breast. J Carcinog. 2011; 10: 31. doi:10.4103/1477-3163.90676 National Cancer Institute. Paget Disease of the Breast. Updated April 10, 2012. National Organization of Rare Diseases. Paget’s Disease of the Breast. Updated 2016. Uthamalingam M & Periyasamy K. Paget’s Disease of Nipple in Male Breast with Cancer. J Clin Diagn Res. 2016 Feb; 10(2): PD14–PD16. doi:10.7860/JCDR/2016/17778.7217.