Paget's disease of the nipple

Symptoms of Paget's disease of the nipple

Paget's disease of the nipple always starts in the nipple and may extend to the areola.

It appears as a red, scaly rash on the skin of the nipple and areola.

The affected skin is often sore and inflamed, and can be itchy or cause a burning sensation. The nipple can sometimes be ulcerated.

The rash is often similar in appearance to other skin conditions, such as:

• eczema
• psoriasis

If you scratch it, or if it's left untreated, it can bleed, become ulcerated or scab over.

If you're experiencing itchiness, burning or bleeding, but the nipple looks normal and isn't red or scaly, it is unlikely to be Paget's disease of the nipple.

You should still have it checked by a doctor.

Around half of all women diagnosed with Paget's disease of the nipple have a lump behind the nipple. In nine out of 10 cases this is an invasive breast cancer.

Invasive cancer is where cancerous cells invade the surrounding breast tissue. Some women with Paget's disease have invasive breast cancer but don't have a lump.

Most women with Paget's disease who don't have a lump have non-invasive cancer. This is where the cancerous cells are contained in one or more areas of the breast and have not spread.

Causes of Paget's disease of the nipple

The cause of Paget's disease of the nipple is unknown. Some women appear to be more at risk of developing breast cancer, but not specifically Paget's disease. There are no known causes that lead to a specific type of breast cancer.

You're more at risk of developing breast cancer if you:

• are older – the risk of developing breast cancer increases with age 
• have close relatives with breast cancer
• have had breast cancer before 
• have had a special type of benign (non-cancerous) breast disease before – certain types of benign breast disease may be associated with an increased risk of breast cancer, but this is rare
• are overweight – particularly if you've been through the menopause
• drink a lot of alcohol

When to see your GP

You should see your GP if you notice any changes in the skin of your nipple or areola.

As Paget's disease of the nipple is associated with breast cancer, the sooner it's diagnosed, the better the outcome is likely to be.

Also see your GP if you develop a lump in your breast. Most breast lumps aren't cancerous but it's important to have them checked.

A biopsy is used to confirm a suspected diagnosis of Paget's disease of the nipple. A small tissue sample will be taken from your nipple and examined to see if it's cancerous.

If the results of the biopsy show Paget's disease, you'll have a mammogram to investigate further.

Treating Paget's disease

Surgery is the main treatment for Paget's disease of the nipple. Depending on whether the cancer has spread, surgery will either involve:

• removing the whole breast (a mastectomy)
• removing the nipple and areola with the breast tissue underneath them (a central excision)

If the whole of your breast is removed, breast reconstruction surgery can be used to create a breast shape to match your remaining breast.

You may need further treatment if you have invasive breast cancer. This may be a combination of:

• chemotherapy 
• radiotherapy
• biological or hormone therapy

If Paget's disease is detected and treated in its early stages, there's a good chance of a full recovery.

Reducing the risk of breast cancer

Changing some lifestyle factors may reduce your risk of developing certain types of cancer, including Paget's disease of the nipple.

These include:

• reducing the amount of alcohol you drink
• exercising regularly

Breast cancer screening is also used to help detect breast cancer early.

The Northern Ireland Breast Screening Programme provides free breast screening every three years for all women aged 50 to 70.

More useful links

• How to use your health services