Paget’s disease of the nipple is a rare type of breast cancer affecting the nipple and the areola (the circle of skin surrounding the nipple).
Paget’s disease causes skin issues like flaking and puckering. It may also cause nipple sensitivity and discharge, too.
Doctors treat Paget's disease of the nipple with surgery and radiation.
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Paget’s disease of the nipple is a rare form of breast cancer that occurs when cancer forms in the nipple and/or the areola.
It’s also known as:
It’s not the same as Paget’s disease of the bone, which isn’t a type of cancer.
Paget’s disease can cause nipple flattening, sensitivity, discharge, scaling, and flaking. It's often mistaken for other skin conditions like eczema.
Most people with Paget’s disease only have it in one breast and often have a cancerous tumor in that breast, too. A person also may have Paget’s disease in one breast and a separate tumor in the opposite breast. In some cases, Paget’s disease shows up on its own.
Doctors don’t “stage” Paget’s disease the way they do other cancers. But if cancer has spread to other breast areas, like your milk ducts, your doctor will stage that cancer.
Paget’s disease is very rare. Between 1% and 4% of all breast cancer cases involve Paget’s disease.
Doctors don’t know what causes Paget’s disease of the nipple. Some experts think cancer cells from other parts of the breast may travel to the nipple and areola. But sometimes Paget’s disease only impacts the nipple or areola.
People who develop it often have other cancerous tumors in the same breast affected by Paget’s disease.
What are Paget’s disease of the nipple risk factors and complications?
Anyone can get Paget's disease of the breast. It mainly impacts women, but men can develop it, too.
Certain factors increase your risk, including:
Left untreated, breast cancer can spread and lead to serious health issues and even death.
Breast cancer treatment can also cause or contribute to other health issues. Chemotherapy and radiation may increase your risk of heart disease, osteoporosis, and high blood pressure. Surgery can sometimes lead to complications like nerve damage.
Your cancer care team will recommend regular checkups after treatment to help you spot and deal with post-treatment complications.
The symptoms of Paget's disease are similar to many skin conditions, which is why it can be confused with dermatitis, psoriasis, or eczema. It's also sometimes mistaken for mastitis, a breast infection that can occur during breastfeeding.
Symptoms of Paget's disease of the nipple include:
It’s always a good idea to see your doctor if you notice changes in your breasts.
But it’s especially important to schedule an appointment if:
When you see your doctor, they'll examine your breast. If they think you may have Paget's disease, they'll recommend tests like a biopsy and mammogram.
Doctors diagnose Paget’s disease of the nipple with a biopsy. They'll remove a small piece of breast tissue with a needle and send it to a lab to see if it contains cancer cells.
Many people with Paget’s disease also have other breast cancer tumors. So, after your biopsy, your doctor will also recommend other tests to check for cancer, too.
They may recommend:
If you’re diagnosed with Paget’s disease, talk to your doctor about your prognosis (or outlook). The five-year survival rate for people with Paget’s disease of the nipple is more than 80%. That’s about the same as the survival rate for people diagnosed with any form of breast cancer.
The goal of treatment for Paget’s disease is to remove as much of the cancer as possible and to prevent cancer cells from spreading.
Doctors treat Paget’s disease with surgery. They may also recommend other cancer treatments, like radiation.
Surgery is almost always used to treat Paget’s disease. Your doctor may recommend breast-conserving surgery, where they remove your nipple and areola but preserve the rest of your breast.
If you have cancer in other parts of your breast, your doctor may remove them during surgery. This procedure is called a lumpectomy.
If your cancer has spread throughout your breast, your doctor may recommend a mastectomy. This surgery removes your entire breast.
Doctors often treat Paget's disease with radiation therapy, which uses high-energy rays to destroy cancer cells. Radiation therapy reduces the odds that cancer will come back.
Doctors also use radiation if your breast cancer has spread to other parts of your body, like your bones.
If you have cancer in areas of your breast or body, your doctor may recommend other treatments. The goal is to kill cancer cells and reduce the odds that cancer spreads.
These treatments may include:
Your recovery from Paget's disease of the nipple will depend on the type of treatment you received. It usually takes several months to recover from surgery.
It can take months to recover from chemotherapy and radiation, too. These treatments may make you tired and weak and cause side effects like nerve damage and swelling (lymphedema).
UPMC offers complete and compassionate care for breast cancer, including Paget’s disease of the nipple.
When you choose UPMC for breast cancer care, you know you're getting:
Last reviewed by Emilia Diego, MD, and Vikram Gorantla, MD on 2024-06-10.
