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​Paget's Disease of the Nipple

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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What Is Paget’s Disease of the Nipple?

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:

  • Mammary Paget disease.
  • Paget’s disease.
  • Paget disease of the breast.

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.

What are the stages of Paget's disease of the nipple?

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.  

How common is Paget's disease of the nipple?

Paget’s disease is very rare. Between 1% and 4% of all breast cancer cases involve Paget’s disease.

What causes Paget’s disease of the nipple?

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?

Paget’s disease of the nipple risk factors

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: 

  • A family history of breast cancer.
  • Being over age 50.
  • Previous nipple issues like itching, scaling, or crusting.
  • Lifestyle habits that increase your risk of any form of breast cancer, such as not exercising, eating a lot of processed foods, and drinking alcohol.  

Complications of Paget’s disease of the nipple

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.

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What Are the Signs and Symptoms of Paget’s Disease of the Nipple?

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: 

  • Crusty, flaky, scaly, or thickened skin on or around the nipple or areola.
  • Itching, tingling, or redness in the nipple or areola.
  • Nipple flattening or a nipple that turns inward. 
  • Nipple or areola dimpling, puckering, or having an orange peel texture.
  • Yellowish or bloody discharge from the nipple. 

When should I see a doctor about my breast symptoms?

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: 

  • Treatment for a skin condition on your breast, such as eczema, isn't working.
  • Treatment for mastitis isn’t working.
  • You’ve noticed any new changes to your nipple or areola, such as scaling or discharge. 

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How Do You Diagnose Paget’s Disease of the Nipple?

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. 

Tests to diagnose Paget’s disease of the nipple

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: 

  • A mammogram, which is an x-ray of the breast. 
  • A 3D mammogram, which uses low-energy x-rays to create 3D images of the breasts.
  • An ultrasound, which uses high-frequency sound waves to detect cancer tumors. Ultrasounds are often used instead of or after a mammogram because they’re helpful in finding cancer in women under 40 and women with dense breast tissue. 
  • An MRI with or without computer-aided detection (CAD). An MRI uses low-energy x-rays to create images of the breast. CAD is software that helps doctors spot tissue problems that a mammogram alone may not show. 

Paget’s disease of the nipple survival rate

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. 

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How Do You Treat Paget’s Disease of the Nipple?

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 for Paget’s disease of the nipple

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.

Radiation for Paget’s disease of the nipple

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. 

Other treatments for Paget’s disease of the nipple

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:  

  • Chemotherapy (chemo) — These anticancer drugs are injected into your vein (intravenously) or taken by mouth (orally). They travel through your bloodstream and help kill cancer cells throughout your body. 
  • Hormone therapy — Some types of breast cancer react to hormones like estrogen and progesterone. Hormone therapy stops hormones from attaching to receptors on cancer cells, helping to stop cancer growth. 
  • HER2 target therapy — These medicines target proteins on cancer cells that can make cancer grow. 

How long does it take to recover after treatment for Paget’s disease of the nipple?

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).

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Why Choose UPMC for Care of Paget’s Disease of the Nipple?

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:

  • Access to cancer experts who can help you at every stage of your diagnosis and treatment.
  • Advanced testing that helps diagnose breast cancer as soon as possible.
  • A personalized treatment plan that focuses on your unique needs. 
  • Research-backed treatments for breast cancer, including chemotherapy, radiation, surgery, and reconstructive breast surgery. 

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Last reviewed by Emilia Diego, MD, and Vikram Gorantla, MD on 2024-06-10.