Overview

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.

Symptoms

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

Be aware of any changes in your breasts. If you feel a lump in your breast, or if you experience itching or skin irritation that persists for more than a month, make an appointment with 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.

Causes

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

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.
  • An inherited gene mutation that increases the risk of breast cancer. 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, but black women are more likely to die of the disease.
  • Alcohol. Drinking large amounts of alcohol increases your risk of developing breast cancer.

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.

Prevention

Making changes in your daily life may help reduce your risk of breast cancer. Try to:

  • Ask your doctor about breast cancer screening. Discuss with your doctor when to begin breast cancer screening exams and tests, such as clinical breast exams and mammograms.

    Talk to your doctor about the benefits and risks of screening. Together, you can decide what breast cancer screening strategies are right for you.

  • Become familiar with your breasts through breast self-exam for breast awareness. Women may choose to become familiar with their breasts by occasionally inspecting their breasts during a breast self-exam for breast awareness. If there is a new change, lumps or other unusual signs in your breasts, talk to your doctor promptly.

    Breast awareness can't prevent breast cancer, but it may help you to better understand the normal changes that your breasts undergo and identify any unusual signs and symptoms.

  • 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.
  • 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 your doctor whether it's OK and start slowly.
  • Limit postmenopausal hormone therapy. Combination hormone therapy may increase the risk of breast cancer. Talk with your doctor about the benefits and risks of hormone therapy.

    Some women experience bothersome signs and symptoms during menopause and, for these women, the increased risk of breast cancer may be acceptable in order to relieve menopause signs and symptoms.

    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 your doctor about healthy strategies to accomplish this. Reduce the number of calories you eat each day and slowly increase the amount of exercise.
  • Choose a healthy diet. Women who eat a Mediterranean diet supplemented with extra-virgin olive oil and mixed nuts may have a reduced risk of breast cancer. The Mediterranean diet focuses mostly on plant-based foods, such as fruits and vegetables, whole grains, legumes and nuts. People who follow the Mediterranean diet choose healthy fats, such as olive oil, over butter and fish instead of red meat.

Breast cancer risk reduction for women with a high risk

If your doctor has assessed your family history and other factors and determined that you may have an increased risk of breast cancer, options to reduce your risk include:

  • Preventive medications (chemoprevention). Estrogen-blocking medications may help reduce the risk of breast cancer. Options include tamoxifen and raloxifene (Evista). Aromatase inhibitors have shown some promise in reducing the risk of breast cancer in women with a high risk.

    These medications carry a risk of side effects, so doctors reserve these medications for women who have a very high risk of breast cancer. Discuss the benefits and risks with your doctor.

  • Preventive surgery. Women with a very high risk of breast cancer may choose to have their healthy breasts surgically removed (prophylactic mastectomy). They may also choose to have their healthy ovaries removed (prophylactic oophorectomy) to reduce the risk of both breast cancer and ovarian cancer.

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

Aug. 04, 2017
References
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Paget's disease of the breast