Paget's (PAJ-its) disease of the breast is a rare form of breast cancer. Paget's disease of the breast starts on the nipple and extends to the dark circle of skin (areola) around the nipple. Paget's disease of the breast isn't related to Paget's disease of the bone, a metabolic bone disease.

Paget's disease of the breast occurs most often in women older than age 50. Most women with Paget's disease of the breast have underlying ductal breast cancer, either in situ — meaning in its original place — or, less commonly, invasive breast cancer. Only in rare cases is Paget's disease of the breast confined to the nipple itself.

Paget's disease of the breast affects your nipple and usually the skin (areola) surrounding it. It's easy to mistake the signs and symptoms of Paget's disease of the breast for skin irritation (dermatitis) or another noncancerous (benign) skin condition.

Possible signs and symptoms of Paget's disease of the breast include:

  • Flaky or scaly skin on your nipple
  • Crusty, oozing or hardened skin resembling eczema on the nipple, areola or both
  • Itching
  • Redness
  • A tingling or burning sensation
  • Straw-colored or bloody nipple discharge
  • A flattened or turned-in (inverted) nipple
  • A lump in the breast
  • Thickening skin on the breast

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

The skin changes may come and go early on, or respond to topical treatment, making it appear as if your skin is healing. On average, women experience signs and symptoms for several months before getting a diagnosis.

When to see a doctor

Check your nipple and areola on both breasts on a regular basis, such as during breast self-exams. If you feel a lump, or if you experience itching or skin irritation that persists for more than a month, see your doctor.

If you're being treated for a skin injury on your breast, and the condition doesn't go away with treatment, make a follow-up appointment with your doctor. You may need a biopsy — a procedure that collects a small tissue sample for microscopic analysis — to evaluate the affected area.

Doctors don't know what causes Paget's disease of the breast. The most widely accepted theory is that the disease results from an underlying ductal breast cancer. The cancer cells from the original tumor then travel through milk ducts to the nipple and its surrounding skin. Another theory is that the disease can develop independently in the nipple.

Risk factors that affect your likelihood of developing Paget's disease of the breast are the same factors that affect your risk of developing any other type of breast cancer.

Some factors that make you more susceptible to breast cancer include:

  • Age. Your chances of developing breast cancer increase as you get older.
  • A personal history of breast cancer. If you've had breast cancer in one breast, you have an increased risk of developing cancer in the other breast.
  • A personal history of breast abnormalities. If you've had lobular carcinoma in situ or atypical hyperplasia, your risk of developing breast cancer is higher. Certain benign breast conditions also are associated with a slightly increased risk.
  • Family history. If you have a mother, sister or daughter with breast or ovarian cancer or both, or even a father or brother with breast cancer, you have a greater chance of developing breast cancer.
  • Genetic predisposition. Defects in one of several genes, especially BRCA1 or BRCA2, put you at greater risk of developing breast cancer as well as ovarian and other cancers. Such defects account for fewer than 1 out of 10 breast cancers.
  • Dense breast tissue. Women with dense breast tissue, as seen on a mammogram, face a higher risk of breast cancer.
  • Radiation exposure. If you received radiation treatments to your chest as a child or young adult to treat another cancer, you're more likely to develop breast cancer later in life.
  • Excess weight. Weighing more than is healthy for your age and height increases your risk of breast cancer — especially after menopause and if you gained weight as an adult.
  • Hormone replacement. Taking estrogen after menopause increases the risk of breast cancer for some women.
  • Race. White women are more likely to develop breast cancer than black or Hispanic women are, but black women are more likely to die of the disease.

Having one or more risk factors doesn't necessarily mean you'll develop breast cancer. Most women with breast cancer have no known risk factors.

If you suspect you have Paget's disease of the breast, your initial appointment might be with your family doctor. Or, when you call for an appointment, you may be referred directly to a doctor who specializes in treating breast conditions.

What you can do

To prepare for your appointment:

  • Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
  • Write down any symptoms you're experiencing. Include even those that may seem unrelated to the reason for which you scheduled the appointment.
  • Write down key personal information. Include any major stresses or recent life changes.
  • Make a list of all medications. Also include vitamins or supplements that you're taking.
  • Consider taking a family member or friend along. Sometimes it can be difficult to remember all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.
  • Write down questions to ask your doctor.

Your time with your doctor is limited, so preparing a list of questions can help you make the most of your time together. Some basic questions to ask include:

  • What is likely causing my symptoms or condition?
  • What are other possible causes for my symptoms or condition?
  • What kinds of tests do I need?
  • Is my condition likely temporary or chronic?
  • What is the best course of action?
  • What are the alternatives to the primary approach that you're suggesting?
  • I have other health conditions. How can I best manage them together?
  • Are there any restrictions that I need to follow?
  • Should I see a specialist? What will that cost, and will my insurance cover it?
  • Are there brochures or other printed materials that I can take with me? What websites do you recommend?
  • What will determine whether I should plan for a follow-up visit?

What to expect from your doctor

Your doctor may ask you questions about:

  • The nature of the skin changes on your nipple
  • Whether you also experience nipple discharge, bleeding, burning or itching
  • If you have any other breast signs and symptoms, such as a breast lump or area of thickening
  • If you have any breast pain
  • How long you've experienced signs and symptoms

Your doctor may also ask questions about your personal and family medical history and other possible risk factors for breast cancer.

Tests to evaluate your condition may include:

  • Clinical breast exam and physical exam. During this exam, your doctor physically checks for unusual areas in both breasts, noting the appearance of the skin on and around your nipples and feeling for any lumps or areas of thickening.
  • Mammography. Mammography — an X-ray exam of your breast tissue — may indicate whether the nipple and skin changes are linked to an underlying breast cancer, as is usually the case in Paget's disease of the breast. If results from mammography in both breasts don't reveal any signs of breast cancer, your doctor may follow up with magnetic resonance imaging (MRI), which may detect cancer that's not able to be seen on a mammogram.
  • Breast biopsy. During a biopsy, your doctor obtains a small sample of tissue from the skin of your nipple for examination under a microscope. If you have nipple discharge, a sample of the discharge might also be collected. If you have a breast lump, a biopsy of that tissue will also be done. If cancer cells are detected in the samples collected, you may be referred to a breast surgeon to discuss treatment options.
  • Sentinel lymph node biopsy. If you have invasive breast cancer, the lymph nodes under your arm (axillary lymph nodes) need to be checked to see if the cancer has spread to this area. This can be done in a procedure known as a sentinel lymph node biopsy. During the biopsy, your surgeon locates the sentinel nodes — the first lymph nodes to receive the drainage from breast tumors and therefore the first place cancer cells will travel. If a sentinel node is removed, examined and found to be normal, the chance of finding cancer in any of the remaining nodes is small and no other nodes need to be removed.

If you have Paget's disease of the breast, you will likely need surgery. The type of surgery depends on the condition of the skin around your nipple and how advanced the underlying cancer is.

Surgical options include:

  • Simple mastectomy. This procedure involves removing your entire breast, but not the lymph nodes in your armpit (axillary lymph nodes). Simple mastectomy might be recommended in cases in which an underlying breast cancer exists but hasn't spread to the lymph nodes.
  • Lumpectomy. Breast-conserving surgery (lumpectomy) involves removing only the diseased portion of your breast. Your surgeon removes your nipple and areola along with a wedge- or cone-shaped section of your breast. The surgeon focuses on removing as little breast tissue as possible, while ensuring that the tissue removed includes an outer margin free of cancer cells so that only healthy cells remain. Lumpectomy to treat Paget's disease of the breast requires follow-up radiation therapy. Lumpectomy wouldn't be recommended if you can't have radiation therapy for some reason. Most women have nipple reconstruction after their treatment.

Adjuvant therapy

After your operation, your doctor may recommend additional treatment (adjuvant therapy) with anti-cancer drugs (chemotherapy), radiation therapy or hormone therapy to prevent a recurrence of breast cancer. Your specific treatment will depend on the extent of the cancer and whether your tumor tests positive for certain characteristics, such as having estrogen or progesterone receptors.

A breast cancer diagnosis can be overwhelming. And just when you're trying to cope with the shock and the fears about your future, you're asked to make important decisions about your treatment.

Every woman finds her own way of coping with a breast cancer diagnosis. Until you find what works for you, it might help to:

  • Learn what you need to know about your breast cancer. If you'd like to know more about your breast cancer, ask your doctor for details — the type, stage and hormone receptor status. Ask for good sources of up-to-date information on your treatment options. Knowing more about your cancer and your options may help you feel more confident when making treatment decisions. Still, some women may not want to know the details of their cancer. If this is how you feel, let your doctor know that, too.
  • Talk with other breast cancer survivors. You may find it helpful and encouraging to talk to other women with breast cancer. Contact the American Cancer Society to find out about support groups in your area. Organizations that can connect you with other cancer survivors online or by phone include the Breast Cancer Network of Strength and CancerCare.
  • Find someone to talk with. Find a friend or family member who is a good listener or talk with a clergy member or counselor. Ask your doctor for a referral to a counselor or other professional who works with cancer survivors.
  • 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, whether it's having someone to talk to if you're feeling low or getting help preparing meals.
  • Maintain intimacy with your partner. In Western cultures, women's breasts are associated with attractiveness, femininity and sexuality. Because of these attitudes, breast cancer may affect your self-image and erode your confidence in intimate relationships. Talk to your partner about your insecurities and your feelings.
  • Take care of yourself. Make your well-being a priority during cancer treatment. Get enough sleep so that you wake feeling rested, choose a diet full of fruits and vegetables, make time for gentle exercise on days you feel up to it, and find time for things you enjoy, such as reading or listening to music. If you need to, be prepared to relinquish your role as caretaker for a while. This doesn't mean you're helpless or weak. It means you're using all your energy to get well.

There are some things you can do to help prevent your chances of developing breast cancer. Some things, however, are beyond your control.

Risk factors that can't be avoided

These include your:

  • Age
  • Genetics
  • Family history

Risk factors that are within your control

Healthy lifestyle habits may help to protect you from breast cancer. These include:

  • Getting enough exercise
  • Limiting alcohol
  • Eating healthy foods
  • Trying to maintain a healthy weight

Medications and surgical procedures

Some interventions may help reduce your chance of developing breast cancer:

  • Preventive medications. Sometimes medication is recommended for prevention, but you may experience side effects.
  • Risk-reducing surgery. Some women decide to pursue prophylactic mastectomy (removal of one or both breasts in hope of preventing or reducing risk of breast cancer). However, this isn't the best option for everyone.

Discuss your personal risk factors with your doctor and ask whether you would be a candidate for treatment that may reduce that risk.

Mar. 27, 2013