Pagets disease of the breast, also known as Pagets disease of the nipple, is a rare type of breast cancer. As its name suggests, it affects the nipple but it is usually not confined just to the nipple. Tissues deeper within the breast are often involved.

Pagets disease of the breast can also affect men, though this is extremely rare. Pagets disease of the breast is not the same as Pagets disease of the bone.

Why it is important to recognise Pagets disease

Signs of Pagets disease are due to changes in the nipple but the problem usually starts deeper within the breast. Recognition of Pagets disease often leads to the discovery of underlying invasive breast cancer.

Women diagnosed with Pagets disease of the breast sometimes also have ductal carcinoma in situ (DCIS). This means that the cells of the breast have changed and started to turn into cancerous cells, but this is not yet cancer. Spotting the symptoms early allows prompt treatment that can deal with the problem before invasive cancer develops.

What are the symptoms of Pagets disease?

Pagets disease begins on the nipple as a red, itchy rash, which can then spread to the areola, the darker area of skin around the nipple. The skin becomes scaly and there can be bleeding, nipple discharge, open sores, and symptoms similar to eczema or psoriasis on the nipple.

Some cases of Pagets disease are misdiagnosed and treated as a skin condition before cancer tests are performed. Pagets disease is therefore often diagnosed relatively late.

What causes Pagets disease of the breast?

The cells from the nipple and from the associated breast tumour have a characteristic appearance when they are viewed under a microscope and are called Paget cells.

The cause of Pagets disease is not fully understood, but the high rate of underlying invasive breast cancer that is found in almost all women with Pagets disease suggests that the Paget cells that develop on the surface of the nipple come from the underlying tumour. As this develops and invades the breast tissue some of the cells migrate to the nipple.

How is Pagets disease of the breast diagnosed?

If you go to your GP with a sore nipple, you will first be given a thorough medical examination. If other causes of your symptoms, such as a skin condition are ruled out, you will then have a biopsy. This is usually done at the same time as an ultrasound scan to make sure that the biopsy sample is taken from an obviously abnormal area.

Some cells may also be scraped from the surface of the nipple for examination under a microscope; this is known as scrape cytology. Cells from the biopsy or scrape are examined to find out if they are Paget cells.

Following diagnosis of Pagets disease, you will then usually have a mammogram to check for underlying breast cancer. If invasive breast cancer is found you will then have more tests, including an ultrasound scan, an MRI scan and blood tests. Further biopsies may be needed to establish the stage and grade of the underlying breast cancer, and whether it has spread to other areas of the body.