Paget’s disease of the breast

Paget's disease of the breast is a rare condition of the nipple that is associated with some breast cancers. It is also known as Paget’s disease of the nipple or mammary Paget’s disease.

What is Paget’s disease of the breast?

This condition develops in the nipple or the darker area of skin around it (the areola). 

Paget’s disease is a sign that there might be breast cancer in the tissues behind the nipple. It is possible for someone to have Paget’s of the breast with no underlying cancer, but this is less common.

About half of the people diagnosed with Paget's disease of the breast have a lump behind the nipple. Most people with a lump behind the nipple are found to have invasive breast cancer.

But most people with Paget’s disease of the breast who have no lump behind the nipple, have ductal carcinoma in situ (DCIS). This means that some cells have started to turn into cancer but they are completely contained within the lining of the breast ducts.

Paget's disease of the breast is rare. It is found in 1 to 3 out of every 100 breast cancers (1 to 3%) diagnosed in women. It can also happen in men but this is even more rare.

Symptoms of Paget’s disease of the breast

It usually first appears as a red, scaly rash of the skin over the nipple and areola. It can be itchy, painful or cause a burning sensation. You might have some discharge or bleeding from the nipple. Or the nipple can change in appearance, going inwards when it wasn’t inverted before.

The rash looks very similar to other skin conditions such as dermatitis or eczema.

While Paget’s disease of the breast can cause these particular symptoms, it’s worth being aware of the general symptoms of breast cancer.

Diagnosing Paget’s disease of the breast

To diagnose Paget’s disease, you usually have a sample of tissue taken from the skin around the nipple. This is a biopsy. Other tests you may have include:

• a mammogram (an x ray of the breast)
• an ultrasound scan of the breast
• a biopsy of the breast lump (if there is a lump)

You may also have an MRI of the breast if the doctors need more information to help confirm whether there is breast cancer or not.

Treatment for Paget’s disease of the breast

The main treatment is surgery. You have surgery to remove just the affected area (breast conserving surgery) or the whole breast (mastectomy).

Breast conserving surgery

For some women, it is possible to remove just the affected area together with a border of healthy tissue. The surgeon usually removes the nipple and areola. You can then have a new nipple made later, usually when you have fully recovered from your treatment. There are different ways to recreate a new nipple, these include:

• having a nipple tattoo
• having a nipple made from your own body tissue or fat
• using a stick on nipple

After surgery, you then have radiotherapy to the rest of the breast.

Mastectomy

Your surgeon may recommend you have a mastectomy if the Paget's disease affects a large area. You may also choose to have a mastectomy if surgery to remove the affected area will not leave you with a good breast shape. You may get a better appearance if you have the whole breast removed and then have surgery to make a new breast shape (breast reconstruction). 

Treatment after surgery

You might have more treatment after surgery if you have Paget’s disease with invasive breast cancer or DCIS. The treatments you might have include:

• chemotherapy
• hormone therapy
• targeted drugs

Follow up

You usually have regular check ups after treatment for Paget’s disease of the breast. How often you have check ups depends on your individual situation.

It’s important to remember that you can contact your doctor or specialist nurse if you notice a new symptom or have questions between your check ups. You can also speak to your GP.

Research and clinical trials

Doctors and researchers are carrying out trials to find better tests and treatments for breast cancer and other conditions such as Paget’s disease.

Coping with Paget's disease of the breast

Coping with breast cancer can be difficult. There is help and support available to you and your family.