Paget's Disease of the Breast: Nipple Changes, Diagnosis and Treatment

What is Paget's disease of the breast?

Paget's disease of the breast, also known as Paget's disease of the nipple, is a rare condition that affects the skin of the nipple. According to the NHS, it usually shows up as a red, scaly rash on the nipple that may spread to the dark area of skin around it (the areola). It is a sign that there may be breast cancer in the breast behind the nipple, although not everyone with Paget's disease has an obvious lump.

It is named after Sir James Paget, the doctor who first described it. It is not the same as Paget's disease of the bone, which is a completely different and unrelated condition that happens to share his name. This guide is only about Paget's disease of the breast.

Cancer Research UK explains that Paget's disease is found in roughly 1 to 3 out of every 100 breast cancers, so it is uncommon. It mostly affects women, but it can also happen in men, which is rarer still. Because it is unusual and looks like an everyday skin problem, it is easy to overlook, which is exactly why it is worth knowing about.

This page is general information, not personal medical advice. If you have noticed a change to your nipple or the skin around it, please see your GP so it can be checked. The sooner any problem is looked at, the better.

What does it look and feel like?

The NHS says the first symptom is usually a red, scaly rash on the nipple. The affected skin is often sore and inflamed, and it can be itchy or cause a burning feeling. Paget's disease almost always starts on the nipple itself and then, after a few weeks, may spread outwards onto the areola.

Other symptoms that people may notice include:

• A rash that crusts, scabs, bleeds or oozes, especially if it is scratched.
• Skin that becomes thickened, flaky or ulcerated (where the skin breaks down).
• A tingling, itching or burning feeling in the nipple area.
• Fluid leaking from the nipple (nipple discharge).
• The nipple turning inwards (becoming inverted) when it was not before.
• Sometimes a lump that can be felt behind or near the nipple.

Paget's disease usually affects only one nipple. It tends not to clear up on its own and does not respond to creams used for skin conditions such as eczema. If a rash or change to your nipple does not get better within a couple of weeks, or keeps coming back, it is important to have it checked rather than carrying on treating it yourself.

How is it different from eczema?

This is one of the most common worries, because Paget's disease and nipple eczema can look very similar. Both can cause a red, scaly, itchy rash. The NHS points out that Paget's disease can be difficult to tell apart from eczema just by looking, which is why people are advised to see a GP rather than assume it is eczema and treat it at home.

There are some clues that doctors look for, although none of them is a substitute for proper testing:

• Where it starts. Paget's disease usually affects the nipple first and then spreads to the areola. Eczema and similar skin conditions more often affect the areola first and then the nipple.
• One side or both. Paget's disease usually affects just one nipple. Nipple eczema more commonly affects both sides.
• How it responds to treatment. Eczema often improves with steroid creams, while Paget's disease does not clear up with these creams.
• Other changes. Paget's disease may come with a lump, nipple discharge, ulceration or a newly inverted nipple, which are not usual features of eczema.

The key message is simple: a rash on the nipple that does not settle, that only affects one side, or that does not respond to eczema creams should always be checked. Most nipple rashes turn out to be harmless, but only an examination and tests can tell for sure. Seeing your GP does not mean you have cancer; it means you are getting the right answer quickly.

The link with breast cancer

Paget's disease of the breast is almost always linked to cancer in the breast behind the nipple, even though the rash itself is on the skin. Understanding this link helps explain why it needs proper investigation rather than just skin treatment.

According to Cancer Research UK and the NHS, about half of people diagnosed with Paget's disease also have a lump in the breast that can be felt. In most of these cases, the lump turns out to be an invasive breast cancer, which means cancer cells have begun to spread into the surrounding breast tissue. In the people who do not have a lump, the underlying problem is usually ductal carcinoma in situ (DCIS), which is a very early, non-invasive cancer where abnormal cells are contained inside the breast ducts and have not spread.

Occasionally, Paget's disease is found without any other cancer in the breast, but this is less common. Because the rash can be the first and only outward sign that something is wrong inside the breast, it is taken seriously and investigated fully. The cause of Paget's disease itself is not fully understood. There is nothing you have done to bring it on.

How Paget's disease is diagnosed

If your GP thinks your nipple changes could be Paget's disease, they will refer you to a breast clinic. The NHS explains that breast clinics often use a 'triple assessment', which means three things together: a doctor examining you, a scan, and taking a small sample of tissue. Using all three gives the most reliable answer.

The tests you may have include:

• A physical examination of both breasts, the nipples and the lymph nodes under the arm.
• A skin biopsy, where a small sample of skin is taken from the nipple or areola and looked at under a microscope. This is the main way to confirm Paget's disease, as it shows the characteristic 'Paget cells'.
• A mammogram, which is a low-dose breast X-ray that can show changes in the breast tissue.
• An ultrasound scan, which uses sound waves to look more closely at the breast and the lymph nodes.
• A breast biopsy if a lump or other change is found, to check whether it is cancer and what type it is.
• Sometimes an MRI scan, which gives detailed pictures and can help show how much of the breast is affected.

Waiting for tests and results can be an anxious time. Your breast care nurse is there to answer questions and support you. It is worth knowing that a referral to a breast clinic is routine, and most people referred with nipple changes do not turn out to have cancer.

How Paget's disease is treated

Treatment depends on what the tests find, particularly whether there is invasive cancer, DCIS, or no other cancer in the breast. The NHS explains that surgery is the main treatment, and your team will recommend the option best suited to your situation.

The main surgical options are:

• Breast-conserving surgery, sometimes called a wide local excision or central excision. This removes the nipple, areola and the affected tissue underneath, while keeping the rest of the breast. It is usually followed by radiotherapy to lower the chance of the cancer coming back.
• Mastectomy, which removes the whole breast. This may be advised if the cancer is more widespread, if there are several areas affected, or if it is your preferred choice.

Some people also have the lymph nodes under the arm checked or removed during surgery, to see whether any cancer has spread there. This is often done with a procedure called a sentinel lymph node biopsy.

After surgery, you may be offered breast reconstruction or nipple reconstruction if you wish, either at the same time or later on. There are several ways to do this, including using your own tissue, an implant, or nipple tattooing to recreate the appearance of a nipple. Your surgeon can explain what is possible for you.

If an invasive cancer or DCIS is found, you may need further treatment alongside surgery. According to the NHS, this can include a combination of radiotherapy, chemotherapy, hormone (endocrine) therapy, or targeted drugs, depending on the exact features of the cancer. Your oncologist will talk you through which treatments are right for you and why.

Outlook, follow-up and getting support

The outlook for Paget's disease depends mainly on whether there is an underlying cancer and, if so, what type and stage it is. As with all breast cancers, finding and treating it early generally gives the best chance of a good outcome. The NHS notes that the sooner Paget's disease is diagnosed, the better the result is likely to be, which is another reason not to delay getting nipple changes checked.

After treatment, you will usually have regular follow-up appointments and may continue to have mammograms to keep an eye on things. Tell your team about any new symptoms between appointments rather than waiting. Many people go on to live full and healthy lives after treatment for Paget's disease.

Living with a breast cancer diagnosis can affect how you feel emotionally as well as physically. Support is available from your breast care nurse, your GP, and UK charities that offer information, helplines and the chance to talk to others who have been through something similar. You do not have to manage it alone.

Knowing the warning signs of breast cancer, including unusual nipple changes, is part of looking after your health. Awareness and timely diagnosis are not always within easy reach for everyone. Our charity works to improve early detection and fair access to checks and treatment for underserved communities, so that more people recognise the signs early and can get help sooner.