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 after age 50. Most people with this diagnosis also have underlying ductal breast cancer, either in situ — meaning in its original place — or, less commonly, invasive breast cancer. Only rarely 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, signs and symptoms occur for several months before a diagnosis is made.

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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.

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:

  • Increasing age. Your risk of breast cancer increases as you age.
  • A personal history of breast conditions. If you've had a breast biopsy that found lobular carcinoma in situ (LCIS) or atypical hyperplasia of the breast, you have an increased risk of breast cancer.
  • 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 family history of breast cancer. If your parent, sibling or child was diagnosed with breast cancer, particularly at a young age, your risk of breast cancer is increased. Still, the majority of people diagnosed with breast cancer have no family history of the disease.
  • Inherited genes that increase cancer risk. Certain gene mutations that increase the risk of breast cancer can be passed from parents to children. The most well-known gene mutations are referred to as BRCA1 and BRCA2. These genes can greatly increase your risk of breast cancer and other cancers, but they don't make cancer inevitable.
  • Radiation exposure. If you received radiation treatments to your chest as a child or young adult, your risk of breast cancer is increased.
  • Obesity. Being obese increases your 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. If you began menopause at an older age, you're more likely to develop breast cancer.
  • Having your first child at an older age. If you give birth to your first child after age 30, you may have an increased risk of breast cancer.
  • Having never been pregnant. If you've never been pregnant, your risk of breast cancer may be increased.
  • Postmenopausal hormone therapy. Hormone therapy medications that combine estrogen and progesterone to treat the signs and symptoms of menopause may increase the risk of breast cancer. The risk decreases when you stop taking these medications.
  • Drinking alcohol. Drinking alcohol increases the risk of breast cancer.

Prevention

Breast cancer risk reduction for people with an average risk

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. You may choose to become familiar with your breasts by occasionally inspecting your breasts during a breast self-exam for breast awareness. If there is a new change, lumps or other unusual signs in your breast, 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. If you choose to drink alcohol, do so in moderation. For healthy adults, that means up to one drink a day for women and up to two drinks a day for men.
  • 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.

    You may experience bothersome signs and symptoms during menopause and, for you, 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. Eating a Mediterranean diet supplemented with extra-virgin olive oil and mixed nuts may reduce your 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 people with a high risk

If your doctor has determined that you have an increased risk of breast cancer, you may discuss options to reduce your risk, such as:

  • Preventive medications (chemoprevention). Estrogen-blocking medications reduce the risk of breast cancer if you have a high risk of the disease.

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

  • Preventive surgery. If you have a very high risk of breast cancer, you may choose to have your healthy breasts surgically removed (prophylactic mastectomy). You may also choose to have your healthy ovaries removed (prophylactic oophorectomy) to reduce the risk of both breast cancer and ovarian cancer.

May 20, 2021
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