Paget's disease of the breast
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What is Paget's disease of the breast?
Paget’s disease of the breast is a condition that causes a scaly rash on the skin of the nipple. This can look like eczema.
Most people who have Paget’s disease also have breast cancer. So it is sometimes called a rare type of breast cancer. The breast cancer may be:
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Ductal carcinoma in situ (DCIS)
DCIS cancer cells are contained inside the milk ducts. It is non-invasive.
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Invasive breast cancer
Invasive breast cancer starts in the ducts or lobules of the breast, and spreads into the surrounding breast tissue.
Paget’s disease affects about 1 to 2 out of every 100 women with breast cancer (1 to 2%).
It is most common in women aged 50 and over. But it can happen at a younger age. It can affect men, but this is very rare.
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Symptoms of Paget's disease
The first symptom of Paget's disease is usually a red scaly, rash on the nipple. This might be harder to see if you have black or brown skin. The skin in the area may look darker than your usual skin tone.
It may also affect the dark area of skin surrounding the nipple (the areola). This can happen after you have had the nipple rash for a few weeks. The rash does not go away, and it may become sore. Paget’s disease usually only affects 1 nipple.
Other symptoms may include:
- the skin of the nipple and areola becoming inflamed
- the skin of the nipple and areola crusting, bleeding and ulcerating (becoming like an ulcer)
- skin thickening on the nipple or areola
- an itching or burning sensation in the area
- fluid (discharge) leaking from the area
- the nipple turning inwards (becoming inverted).
There may or may not be a lump in the breast. About half of women with Paget’s disease (50%) have a breast lump that they can feel at diagnosis.
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Causes of Paget's disease
Doctors do not know what causes Paget's disease. The risk factors are similar to those for DCIS and invasive breast cancer.
Diagnosis of Paget's disease
It may be possible to confuse Paget’s disease with other skin conditions such as eczema, dermatitis and psoriasis. This is because they look similar. Paget’s disease can be difficult to diagnose just by looking at it.
Paget’s disease usually affects the nipple first. After a few weeks, it spreads to the areola. Other skin conditions usually affect the areola first and then the nipple.
Tests for Paget's disease
Your first test will usually be to check the skin changes on your nipple. Your specialist breast cancer doctor may do the following tests:
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A skin or punch biopsy
The doctor removes a small sample of skin from the affected nipple and tissue from under the skin. They inject some local anaesthetic into the skin to numb the area first. The sample is looked at under a microscope for cancer cells.
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A nipple scrape
The doctor gently scrapes or presses cells from the skin of the affected nipple onto a glass slide. These are looked at under a microscope.
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A core biopsy through the nipple
The doctor removes a core of skin from the nipple and a small piece of tissue from the breast ducts underneath. They inject some local anaesthetic into the skin to numb the area first.
As well as some of these tests, you usually have the same tests you have for any other type of breast cancer. You usually have the following scans:
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A mammogram
A mammogram is an x-ray of the breast.
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An ultrasound scan
An ultrasound uses sound waves to produce a picture of the breast tissue and the lymph nodes (glands) in the armpit.
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An MRI scan of the breast
An MRI uses magnetism to build up a detailed picture of the breast area.
Breast biopsy
You will also have a biopsy. This is when your cancer doctor takes small samples of cells or tissue from your breast. You will have an injection of local anaesthetic to numb the area first.
The samples are looked at under a microscope to check for cancer cells. This is to find out whether there are any DCIS or invasive breast cancer cells.
If invasive breast cancer cells are found, you doctor will arrange other tests. This is to find out whether the cancer cells have receptors (proteins) for hormones or for HER2.
Your cancer doctor and breast care nurse will explain which type of biopsy is best for you.
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Staging and grading of Paget's disease
Stage
The stage of a cancer describes its size and how far it has spread. Knowing this helps your cancer doctor plan the best treatment for you.
DCIS is the earliest stage of breast cancer. This is when changes are happening to cells which might become breast cancer. Invasive breast cancer is usually staged using a number system from 1 to 4.
Grade
The grade of breast cancer is how the cancer cells look under a microscope. The grade gives an idea of how slowly or quickly the cancer may develop.
Treatment for Paget's disease
The main treatment for Paget’s disease is surgery. The type of operation you have depends on:
- the size of the area of Paget’s disease
- whether there is DCIS or invasive breast cancer.
- where the affected area is in the breast.
Some people may have other treatments after surgery. For example, you may have radiotherapy to the breast.
Further treatment will depend on whether the cancer is DCIS or invasive breast cancer. Your cancer doctor or nurse will explain more about your treatment options.
Macmillan is also here to support you. If you would like to talk, you can:
- call the Macmillan Support Line for free on 0808 808 00 00.
- Chat to our specialists online.
- Visit our breast cancer forum to talk with people who have been affected by breast cancer including Paget's disease of the breast, share your experience, and ask an expert your questions.
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About our information
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References
Below is a sample of the sources used in our Paget’s disease of the breast information. If you would like more information about the sources we use, please contact us at cancerinformationteam@macmillan.org.uk
Sabel, M.S. Weaver, D.L. Paget disease of the breast (PDB).Uptodate. [Internet] Available from: https://www.uptodate.com/contents/paget-disease-of-the-breast-pdb [accessed July 2021]
Yasir M, Khan M, Lotfollahzadeh S. Mammary Paget Disease. Updated 2023. StatPearls Publishing; 2024. Available from: https://www.ncbi.nlm.nih.gov/books/NBK563228/
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Reviewers
This information has been written, revised and edited by Macmillan Cancer Support’s Cancer Information Development team. It has been reviewed by expert medical and health professionals and people living with cancer. It has been approved by Senior Medical Editor, Dr Rebecca Roylance, Consultant Medical Oncologist.
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