Paget's disease of the breast is a rare form of breast cancer that affects the nipple. Paget's disease of the breast often causes scaly or crusty skin on the nipple. The affected area may slowly grow to involve the skin around the nipple, called the areola.

Paget's is pronounced PAJ-its. Paget's disease of the breast also is called mammary Paget's disease.

Paget's disease of the breast refers to breast cancer in the nipple. But most people with this condition also have breast cancer in the breast tissue. If your healthcare team thinks you have Paget's disease of the breast, you'll likely have tests to look for cancer in other parts of the breast.

Treatment for Paget's disease of the breast is similar to treatment for other forms of breast cancer. Treatments may include surgery, radiation therapy and medicines.

Paget's disease of the breast isn't related to Paget's disease of the bone, which is a metabolic bone disease.

Symptoms

Signs and symptoms of Paget's disease of the breast include:

• Flaky or scaly skin on the nipple.
• Crusty, oozing or hardened skin that looks like eczema on the nipple, areola or both.
• Itching.
• A burning sensation.
• Straw-colored or bloody nipple discharge.
• A turned-in nipple.
• A lump in the breast.
• Thickening skin on the breast.

Symptoms usually occur in one breast only. The symptoms typically start in the nipple and may spread to the areola and other areas of the breast.

When to see a doctor

Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you.

Causes

It's not clear what causes Paget's disease of the breast. It also isn't clear where this breast cancer starts. One theory is that Paget's disease of the breast starts in the breast tissue. Breast cancer might form in the breast tissue and then spread through the milk ducts to the nipple. Another theory is that Paget's disease of the breast starts in the nipple.

Despite the questions about how Paget's disease of the breast happens, healthcare professionals know that it starts with DNA changes. All breast cancers start when breast cells develop changes in their DNA. A cell's DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA tells the cells to grow and multiply at a set rate. The DNA also tells the cells to die at a set time.

In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to grow and multiply quickly. Cancer cells can keep living when healthy cells would die. This causes too many cells. In Paget's disease of the breast, the cancer cells build up in the nipple.

Risk factors

Paget's disease of the breast is a form of breast cancer. Factors that may increase the risk of breast cancer include:

• A family history of breast cancer. If a blood relative, such as a parent, sibling or child, had breast cancer, your risk of breast cancer is increased. The risk is higher if your family has a history of getting breast cancer at a young age. The risk also is higher if you have multiple blood relatives with breast cancer. Still, most people diagnosed with breast cancer don't have a family history of the disease.
• A personal history of breast cancer. If you've had cancer in one breast, you have an increased risk of getting cancer in the other breast.
• A personal history of breast conditions. Having certain breast conditions raises your risk of breast cancer. These conditions include lobular carcinoma in situ, also called LCIS, and atypical hyperplasia of the breast. If you've had a breast biopsy that found one of these conditions, you have an increased risk of breast cancer.
• Beginning your period at a younger age. Beginning your period before age 12 increases your risk of breast cancer.
• Beginning menopause at an older age. Beginning menopause after age 55 increases the risk of breast cancer.
• Dense breast tissue. Breast tissue is made up of fatty tissue and dense tissue. Dense tissue is made of milk glands, milk ducts and fibrous tissue. If you have dense breasts, you have more dense tissue than fatty tissue in your breasts. Having dense breasts can make it harder to detect breast cancer on a mammogram. If a mammogram shows that you have dense breasts, you have an increased risk of breast cancer. Talk with your healthcare team about other tests you might have in addition to mammograms to look for breast cancer.
• Drinking alcohol. Drinking alcohol increases the risk of breast cancer.
• Female sex assigned at birth. Women are much more likely than men are to get breast cancer. But everyone is born with some breast tissue, so anyone can get breast cancer.
• Having your first child at an older age. Giving birth to your first child after age 30 may increase the risk of breast cancer.
• Having never been pregnant. Never having been pregnant increases the risk of breast cancer. Having been pregnant one or more times lowers the risk.
• Increasing age. The risk of breast cancer goes up as you get older.
• Inherited DNA changes that increase cancer risk. Certain DNA changes that increase the risk of breast cancer can be passed from parents to children. The most well-known changes are BRCA1 and BRCA2. These changes can greatly increase your risk of breast cancer and other cancers, but not everyone with these DNA changes gets cancer.
• Hormone therapy for menopause. Taking certain hormone therapy medicines to control the symptoms of menopause may increase the risk of breast cancer. The risk is linked to hormone therapy medicines that combine estrogen and progesterone. The risk goes down when you stop taking these medicines.
• Obesity. People with obesity have an increased risk of breast cancer.
• Radiation exposure. If you received radiation treatments to your chest as a child or young adult, your risk of breast cancer is higher.

Prevention

Making changes in your daily life may help reduce your risk of Paget's disease of the breast and other forms of breast cancer. Try to:

• Ask about breast cancer screening. Talk with your healthcare professional about when to begin breast cancer screening. Ask about the benefits and risks of screening. Together, you can decide which breast cancer screening tests are right for you.

• Become familiar with your breasts. You may choose to become familiar with your breasts by occasionally inspecting them during a breast self-exam for breast awareness. If there is a new change, a lump or something not typical in your breasts, report it to a healthcare professional right away.

  Breast awareness can't prevent breast cancer, but it may help you to better understand the look and feel of your breasts. This might make it more likely that you'll notice if something changes.

• Drink alcohol in moderation, if at all. Limit the amount of alcohol you drink to no more than one drink a day if you choose to drink. For breast cancer prevention, there is no safe amount of alcohol. So if you're very concerned about your breast cancer risk, you may choose to not drink alcohol.
• Exercise most days of the week. Aim for at least 30 minutes of exercise on most days of the week. If you haven't been active lately, ask a healthcare professional whether it's OK and start slowly.

• Limit hormone therapy for menopause. Combination hormone therapy may increase the risk of breast cancer. Talk with a healthcare professional about the benefits and risks of hormone therapy.

  Some people have symptoms during menopause that cause discomfort. These people may decide that the risks of hormone therapy are acceptable in order to get relief. To reduce the risk of breast cancer, use the lowest dose of hormone therapy possible for the shortest amount of time.

• Maintain a healthy weight. If your weight is healthy, work to maintain that weight. If you need to lose weight, ask a healthcare professional about healthy ways to lower your weight. Eat fewer calories and slowly increase the amount of exercise.

Diagnosis

To diagnose Paget's disease of the breast, a healthcare professional might start with a physical exam. The health professional also may ask about your symptoms. Most people with Paget's disease of the breast have cancer within the breast tissue. So you may have imaging tests to look at the breast tissue for signs of cancer. To confirm whether there is cancer, a sample of tissue is removed from the breast for testing.

Breast exam

During a clinical breast exam, a healthcare professional looks at the breasts for anything that's not typical. For Paget's disease of the breast, this might include changes in the skin or to the nipple. Then the health professional feels the breasts for lumps. The health professional also feels along the collarbones and around the armpits for lumps.

Mammogram

A mammogram is an X-ray of the breast tissue. Mammograms are commonly used to screen for breast cancer. If a healthcare professional is concerned that you might have breast cancer, that person might have you do a more detailed mammogram, called a diagnostic mammogram. It's often used to look closely at both breasts.

Breast ultrasound

Ultrasound uses sound waves to make pictures of structures inside the body. A breast ultrasound may give your healthcare team more information about a breast lump. For example, an ultrasound might show whether the lump is a solid mass or a fluid-filled cyst. The healthcare team uses this information to decide which tests you might need next.

Breast magnetic resonance imaging

Magnetic resonance imaging, also called MRI, uses a magnetic field and radio waves to create pictures of the inside of the body. A breast MRI can make more-detailed pictures of the breast. Sometimes this method is used to look closely for any other areas of cancer in the affected breast. It also might be used to look for cancer in the other breast. Before a breast MRI, you usually receive an injection of dye. The dye helps the tissue show up better in the images.

Biopsy

A biopsy is a procedure to remove a sample of tissue for testing in a lab. For Paget's disease of the breast, the biopsy procedure often involves taking a sample of skin. A healthcare professional might use a cutting tool to remove some skin from the affected nipple.

If imaging tests find something worrying in the breast tissue, a healthcare professional might remove tissue from the breast for testing. To get the sample, a healthcare professional puts a needle through the skin and into the breast tissue. The health professional guides the needle using images created with X-rays, ultrasound or another type of imaging. Once the needle reaches the right place, the health professional uses the needle to draw out tissue from the breast. Often, a marker is placed in the spot where the tissue sample was removed. The small metal marker shows up on imaging tests. The marker helps your healthcare team monitor the area of concern.

Treatment

Treatment for Paget's disease of the breast often involves surgery. Other treatments may include radiation therapy and medicines, such as chemotherapy and hormone therapy. Your treatment options may depend on whether the cancer only affects the nipple or whether cancer also is in the breast tissue.

Surgery

Operations used to treat Paget's disease of the breast include:

• Removing the breast cancer. Breast-conserving surgery, also called lumpectomy, involves removing the cancer from the breast. For Paget's disease of the breast, the surgeon might remove the nipple, areola and other parts of the breast that have cancer. Most people who have this surgery also have radiation therapy.
• Removing all of the breast tissue. A mastectomy is surgery to remove all breast tissue from a breast. The most common mastectomy procedure is total mastectomy, also called simple mastectomy. This procedure removes all of the breast, including the lobules, ducts, fatty tissue and some skin, including the nipple and areola.
• Removing a few lymph nodes. A sentinel node biopsy is an operation to take out some lymph nodes for testing. When breast cancer spreads, it often goes to the nearby lymph nodes first. To see if the cancer has spread, a surgeon removes some of the lymph nodes near the cancer. If no cancer is found in those lymph nodes, the chance of finding cancer in any of the other lymph nodes is small. No other lymph nodes need to be removed.
• Removing several lymph nodes. Axillary lymph node dissection is an operation to remove many lymph nodes from the armpit. Your breast cancer surgery might include this operation if imaging tests show that the cancer has spread to the lymph nodes. It also might be used if cancer is found in a sentinel node biopsy.
• Removing both breasts. Some people who have cancer in one breast may choose to have their other breast removed even if it doesn't have cancer. This procedure is called a contralateral prophylactic mastectomy. It might be an option if you have a high risk of getting cancer in the other breast. The risk might be high if you have a family history of cancer or have DNA changes that increase the risk of cancer. Most people with breast cancer in one breast do not get cancer in the other breast.

Complications of breast cancer surgery depend on the procedures you choose. All operations have a risk of pain, bleeding and infection. Removing lymph nodes in the armpit carries a risk of arm swelling, called lymphedema.

You may choose to have breast reconstruction after mastectomy surgery. Breast reconstruction is surgery to restore shape to the breast. Options might include reconstruction with a breast implant or reconstruction using your own tissue. Consider asking your healthcare team for a referral to a plastic surgeon before your breast cancer surgery.

Other treatments

After surgery, your healthcare team may recommend additional treatments. These treatments may help lower the chances that the cancer will come back. Whether you need these other treatments may depend on your cancer.

Other treatments for Paget's disease of the breast may include:

• Radiation therapy.
• Chemotherapy.
• Hormone therapy.

Coping and support

Some people who have been diagnosed with Paget's disease of the breast say their diagnosis felt overwhelming at first. It can be stressful to feel overwhelmed at the same time you need to make important decisions about your treatment. In time, you'll find ways to cope with any feelings you have. Until you find what works for you, here are some ideas that might help.

Learn enough about your cancer to make decisions about your care

If you'd like to know more about your breast cancer, ask your healthcare team for the details of your cancer. Ask for good sources of information where you can learn more about your treatment options. As you learn more about your condition, you might feel more confident about making decisions about your care.

Talk with other breast cancer survivors

You may find it helpful and encouraging to talk with others who have been diagnosed with breast cancer. Contact a cancer support organization in your area to find out about support groups near you or online. In the United States, you might start with the American Cancer Society.

Find someone to talk with about your feelings

Find a friend or family member who is a good listener. Or talk with a clergy member or counselor. Ask your healthcare team for a referral to a counselor or other mental health professional who works with people who have cancer.

Keep your friends and family close

Your friends and family can provide a crucial support network for you during your cancer treatment.

As you begin telling people about your breast cancer diagnosis, you'll likely get many offers for help. Think ahead about things you may want help with. Examples include listening when you want to talk or helping you with preparing meals.

Preparing for your appointment

Make an appointment with a doctor or other healthcare professional if you have symptoms that worry you. If your healthcare professional thinks you might have Paget's disease of the breast, that person may refer you to a specialist. This may be a doctor who treats breast conditions or a doctor who treats cancer.

Appointments can be short and being prepared can help. Here's some information that may help you get ready.

What you can do

When you make the appointment, ask if there's anything you need to do in advance, such as fasting before having a specific test. Make a list of:

• Your symptoms, including any that seem unrelated to the reason for your appointment.
• Key personal information, including major stresses, recent life changes and family medical history.
• All medicines, vitamins and other supplements you take, including the doses.
• Questions to ask your healthcare team.

Take a family member or friend along, if possible, to help you remember the information you're given.

For Paget's disease of the breast, some basic questions to ask include:

• Do I have cancer?
• Do I need more tests?
• What are my treatment options?
• What are the potential risks of these treatment options?
• Do any of the treatments cure my cancer?
• Can I have a copy of my pathology report?
• How much time can I take to consider my treatment options?
• Are there brochures or other printed materials that I can take with me? What websites do you recommend?
• What would happen if I chose not to have treatment?

Don't hesitate to ask other questions.

What to expect from your doctor

Your healthcare professional is likely to ask you several questions, such as:

• What skin changes have you noted in your nipple?
• Have you experienced any nipple discharge, bleeding, burning or itching?
• Do you have any other breast symptoms, such as a breast lump or area of thickening?
• Have you experienced any breast pain?
• When did your symptoms begin?
• What, if anything, seems to make your symptoms better?
• What, if anything, seems to worsen your symptoms?