What is Paget's disease of the breast?

Paget’s disease of the breast is a condition that causes an eczema-like change to the skin of the nipple.

9 out of 10 women who have Paget’s disease (90%) will also have breast cancer. The breast cancer may be:

  • Ductal carcinoma in situ (DCIS)

    DCIS cancer cells are contained inside the milk ducts (non-invasive).

  • Invasive breast cancer

    Invasive breast cancer starts in the ducts or lobes 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 or over. But can happen at a younger age. It can affect men, but this is extremely rare.

Symptoms of Paget's disease

The first symptom of Paget's disease is usually a scaly, red rash affecting the nipple. 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.

Other symptoms may include:

  • the skin of the nipple and areola becoming inflamed
  • the skin of the nipple and areola crusting, bleeding and ulcerating (so it looks like an ulcer)
  • an itching or burning sensation in the area
  • some 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 you can feel at diagnosis.

Causes of Paget's disease

We do not know what causes Paget's disease. The risk factors for DCIS and for invasive breast cancer are similar.

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 and can make Paget’s disease difficult to diagnose by just looking at it.

Paget’s disease usually affects the nipple first, and after a while spreads to the surrounding tissue. But other skin conditions usually affect the areola first and then the nipple.

Most tests you have are the same as for any type of breast cancer.

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:

  • A skin or punch biopsy

    They remove a small piece of skin from the affected nipple and 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.

  • A nipple scrape

    They scrape or press cells from the skin of the affected nipple onto a glass slide. These are looked at under a microscope.

  • A core biopsy through the nipple

    They remove a core of skin from the nipple and a small piece of tissue from the breast ducts underneath.

Apart from the tests above, you usually have the same tests as those for any other type of breast cancer. You usually have scans, which include:

  • A mammogram

    A mammogram is an x-ray of the breast.

  • An ultrasound scan

    An ultrasound uses sound waves to produce a picture of the breast tissue and the lymph nodes (glands) in the armpit.

  • An MRI scan of the breast

    An MRI uses magnetism to build a detailed picture of the breast area.

Breast biopsy

You will also have a biopsy. Your cancer doctor or breast care nurse takes small samples of cells or tissue from your breast.

The samples are looked at under a microscope to check for cancer cells. This is to find out if there are any DCIS or invasive breast cancer cells.

They also do other tests to find out if the cancer cells have receptors (proteins) for hormones, or for HER2.

Your cancer doctor and breast care nurse will explain the type of biopsy that is best for your situation.

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 always the earliest form of breast cancer. Invasive breast cancer is usually staged using a number system that groups it 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. Having further treatment depends on whether the cancer is DCIS or an invasive breast cancer.

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