Paget's disease is a rare skin condition 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 1 to 4 out of every 100 breast cancers (1 to 4%). It can happen in men but this is even more rare.
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. It is sometimes diagnosed quite late, and this may be because it is often first treated as eczema, before any cancer tests are done.
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
Paget's disease is diagnosed from a biopsy. A breast surgeon takes a sample of the affected skin tissue (a biopsy). They send it to the laboratory for examination under a microscope. If the biopsy shows Paget's disease, you then have a mammogram or breast ultrasound, or sometimes an MRI scan.
Treatment for Paget’s disease
You have surgery to remove either the whole breast or just the affected area. 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 biological therapies
For some women, it is possible to remove just the area containing the cancer, together with a border of healthy tissue. You might then have radiotherapy to the rest of the breast.
Removal of the whole breast (mastectomy) may be the only option if:
- a large area is affected by Paget's disease
- there is an area of invasive breast cancer behind the nipple
- your cancer 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. Then you can have surgery to make a new breast shape (breast reconstruction).
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 biological 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. And it reduces the chance of getting a new cancer in the other breast.
Your doctor might recommend you also have biological therapy if your cancer has receptors for biological therapy drugs.
Your doctor might recommend further treatment with chemotherapy if there is a significant 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.
You have regular check ups once you finish your treatment. At your check up appointments your doctor or specialist nurse examines you and asks about your general health.
This is your chance to ask questions and to tell your doctor if anything is worrying you.
It’s important to remember that you can still contact someone if you are worried about a symptom between appointments. Don’t just leave it. Speak to your GP, or contact your consultant or specialist nurse.
How often you have check ups depends on your individual situation.
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.