Paget’s disease of the breast

Paget's disease is a rare skin condition of the nipple that is associated with some breast cancers.

What Paget’s disease of the breast is

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 have a lump behind the nipple. In 9 out of 10 cases, this is an invasive breast cancer. Invasive breast cancer can also be present in some people with Paget's disease who have no lump.

But most people with no lump have carcinoma in situ. This means that there are cancer cells in the biopsy but they are completely contained within the lining of the breast ducts.

Paget's disease is rare. It is found in around 1 to 4 out of every 100 breast cancers (around 1 to 4%). It can happen in men but this is even more rare.

Symptoms

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.

If it isn't treated, or if you scratch it, it can bleed, ulcerate and may scab over. It looks very similar to other skin conditions such as psoriasis 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

To diagnose Paget’s disease, you usually have a biopsy of the skin around the nipple. Other tests may include:

• a mammogram (an x ray of the breast)
• an ultrasound scan of the breast
• a biopsy of the breast tissue (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

You have surgery to remove either the whole breast (mastectomy) or just the affected area (breast conserving surgery). Further treatment depends on:

• whether you have DCIS or a cancer
• the stage (if you have a cancer)
• how abnormal the cells look under the microscope (the grade)
• whether the cancer cells have receptors for particular hormones
• whether the cells have receptors for targeted cancer drugs

Surgery

For some women, it is possible to remove just the area containing the cancer or DCIS, together with a border of healthy tissue. You might then have radiotherapy to the rest of the breast.

Removal of the whole breast (mastectomy) might be the only option if:

• the Paget's disease affects a large area
• there is an area of invasive breast cancer behind the nipple
• your cancer or DCIS is in the central area of the breast

In these situations, surgery to remove just the lump might not leave you with a good breast shape. You may get a better appearance if you have the whole breast removed and have surgery to make a new breast shape (breast reconstruction). 

The surgeon removes the nipple and darker area around the nipple during surgery. You can have a new nipple made later, usually when you have fully recovered from your surgery. There are different ways to recreate a new nipple, these include:

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

When you have your surgery, the surgeon might take out some of the lymph nodes under your arm. They test the nodes to see if they contain cancer cells. This helps to stage the cancer and decide on further treatment.

Hormone therapy and targeted drug therapy

If you have breast cancer and the cells have oestrogen receptors your doctor might suggest hormone therapy. This reduces the risk of the cancer coming back in the same breast (or in the chest wall after mastectomy).

Hormone therapy lowers the risk of the cancer cells spreading anywhere else in the body. It also reduces the chance of getting a new cancer in the other breast.

Your doctor might recommend that you also have targeted drug therapy if your cancer has receptors for targeted cancer drugs.

Chemotherapy

Your doctor might recommend further treatment with chemotherapy if there is a high risk that the cancer might come back. They may suggest this if:

• cancer cells were found in your lymph nodes
• you had a large breast tumour
• your cancer cells were high grade (grade 3)

Giving chemotherapy helps to lower the risk of the cancer coming back in the future.

Follow up

After treatment you usually have regular check ups. At the check ups your doctor or a breast care nurse will examine you and ask about your general health. This is your chance to ask questions and to tell them if anything is worrying you.

How often you have check ups depends on your individual situation but they might go on for at least 5 years. This might include yearly mammograms. 

It’s important to remember that you can contact your doctor or nurse between appointments if you are concerned about a symptom or have questions. You don't have to wait for your next appointment. You can also speak to your GP.

In some hospitals you don't have regular appointments after treatment. But if you have new symptoms or are worried about anything you can phone your doctor or breast care nurse or make an appointment to see them. 

UK guidelines say that everyone who has had treatment for early breast cancer should have a copy of a written care plan. The care plan has information about tests you will have, and signs and symptoms to look out for. It will also include contact details for specialist staff, such as your breast care nurse.

Research and clinical trials

Doctors and researchers are carrying out trials to find better tests and treatments for Paget's disease, ductal carcinoma in situ and breast cancer.