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Macmillan and Cancerbackup merged in 2008. Together we provide free, high quality information for people affected by cancer through our publications, website and phone service. Find out more|.
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This information is about a condition called Paget’s disease of the breast. It should ideally be read with our general information about breast cancer| and ductal carcinoma in situ (DCIS|).
Paget’s disease of the breast| is an eczema-like change in the skin of the nipple, and 9 out of 10 women who have it (90%) have an underlying breast cancer. The underlying breast cancer may be an invasive breast cancer or ductal carcinoma in situ (DCIS). In DCIS, the cancer cells are completely contained within the milk ducts.
Paget’s disease affects about 1–2 out of every 100 women with breast cancer (1–2%). It's most common in women in their 50s, but can occur at a younger or older age. It can affect men, but this is extremely rare.
The cause of Paget's disease is unknown, but certain women seem to be at a higher risk of developing breast cancer. This includes women who have never had children or had them late in life, women who started their periods at a young age or who had a late menopause, and women who have a strong family history| of breast cancer.
The first symptom is usually a scaly, red rash affecting the nipple and sometimes the dark area of skin surrounding the nipple (the areola). The rash always affects the nipple first and may then affect the areola. It doesn't go away and may become sore.
The skin of the nipple and areola may be inflamed. There may also be crusting, bleeding and ulceration. Some women have an itching or burning sensation. Fluid (discharge) may leak from the abnormal area of cells. The nipple may turn inwards (be inverted). There may or may not be a lump in the breast.
Around half of all women who have Paget’s disease have a breast lump that can be felt at the time the disease is diagnosed.
Paget’s disease can be confused with other skin conditions such as eczema, dermatitis and psoriasis, because they can look very similar. This can make Paget’s disease difficult to diagnose.
Paget’s disease usually affects the nipple first and then the surrounding tissue. Whereas other skin conditions usually affect the areola (the dark area of skin around the nipple) first and then spread into the nipple.
Several tests may be carried out to diagnose Paget’s disease of the breast. They may include the following:
Mammograms| may be used to look for changes in the affected breast and to check the other breast.
A gel is spread on the breast and a small device that emits sound waves is rubbed over the area. The echoes are converted into a picture of the breast tissue by a computer. This test is painless and takes just a few minutes.
Treatment depends on:
Surgery| is the main treatment for Paget’s disease. This may involve an operation to remove all of the breast (a mastectomy|) or an operation to remove the affected area of breast, including the nipple, areola and some surrounding normal breast tissue (breast-conserving surgery|).
Some of the lymph nodes in the underarm may also be removed.
A mastectomy may be recommended if the cancer is affecting a wide area, if it's close to the nipple, or if there's DCIS in a number of areas in the breast (multifocal). It may be possible to have breast reconstruction| at the same time as a mastectomy or as a second operation some months later.
Breast‑conserving surgery may be possible if the cancer, or DCIS is close to the nipple and only affecting a small area of breast tissue.
Some people don't need any further treatment after surgery. Others may have radiotherapy, hormonal therapy or chemotherapy. These treatments may be given separately or together.
Radiotherapy| treats cancer by using high-energy x-rays to destroy the cancer cells, while doing as little harm as possible to the healthy cells. Radiotherapy to the remaining breast tissue is usually recommended after breast-conserving surgery.
Some breast cancer cells have oestrogen receptors on their surfaces. This is known as oestrogen receptor positive (ER+) breast cancer. This means that the cells rely on the hormone oestrogen to grow. Oestrogen is a female hormone that's naturally produced in the body.
If you have ER+ breast cancer, you'll be prescribed hormonal therapy| to counteract the effects of oestrogen. There are different types of hormonal therapy, which work in different ways. Some hormonal therapies reduce the amount of oestrogen made by the body. Others stop oestrogen from reaching the cancer cells.
Your doctor or breast care nurse will give you more information about the hormonal therapy you'll be having and any possible side effects it may have.
Chemotherapy| is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It's not used to treat an underlying DCIS, but it may be given if the cancer is invasive.
Herceptin| is one of a new group of drugs called monoclonal antibodies|.
Some breast cells divide and grow when a protein (human epidermal growth factor) attaches itself to another protein called HER2. Herceptin blocks this process by attaching itself to the HER2 protein so that the epidermal growth factor can't reach the breast cancer cells.
Herceptin only works in people who have high levels of the HER2 protein|, which you can be tested for. It can be used to treat early breast cancer or breast cancer that's spread (secondary breast cancer|).
Research into treatments for Paget’s disease of the breast is ongoing. Cancer doctors use clinical trials| to assess new treatments. Before any trial is allowed to take place, an ethics committee must approve it and agree that it's in the interest of patients.
You may be invited to take part in a clinical trial. Your doctor must discuss the treatment with you so that you have a full understanding of the trial and what it involves. You may decide not to take part, or to withdraw from a trial at any stage. You'll then receive the best standard treatment available.
You may have many different emotions|, including anger, resentment, guilt, anxiety and fear. These are all normal reactions and are part of the process many people go through in trying to come to terms with their illness. You may find it helpful to talk things over with your doctor or nurse, or with one of our cancer support specialists|. Family members and close friends can also offer support.
This section has been compiled using information from a number of reliable sources, including:
For answers, support or just a chat, call the Macmillan Support Line free (Monday to Friday, 9am-8pm)
If you have any questions about cancer, need support or just want someone to talk to, ask Macmillan.