Paget's disease of the nipple

Paget’s disease of the nipple, also known as Paget’s disease of the breast, is a rare type of breast cancer.

The term Paget’s disease of the nipple is used to distinguish the condition from Paget’s disease of the bone, which occurs when the normal cycle of bone growth is disrupted, leading to the bones becoming weak and deformed.

A similar type of skin cancer can also occur in other parts of the body.

The rest of this section will use the term Paget’s disease to refer to Paget’s disease of the nipple or breast.

Symptoms of Paget's disease

Paget's disease usually affects the skin of one nipple and produces eczema-like symptoms, appearing as an itchy, red rash on the nipple that can extend to the darker area of surrounding skin (the areola).

It can also appear as a small ulcer or dry, red, flaky patches of skin similar to psoriasis.

Other possible symptoms include:

• itchiness or a burning sensation – alongside visible changes to the nipple
• bleeding from the skin of the nipple

If you're experiencing itchiness, burning or bleeding but the nipple looks normal and isn't red, dry or scaly – this is extremely unlikely to be Paget's disease but should still be checked by a doctor.

Causes of Paget's disease

Paget’s disease is usually a sign of breast cancer in tissue behind the nipple, or breast tissue away from the nipple.

The breast cancer can either be:

• invasive – where cancerous cells invade the surrounding breast tissue
• non-invasive – where the cancerous cells are contained in one or more areas of the breast and are unable to spread

In a small amount of cases, the only evidence of Paget's disease is the nipple changes.

In about half of all cases of Paget’s disease of the nipple, a lump is found in the breast. The majority of people with a lump will have invasive breast cancer, although this does not necessarily mean it has spread.

Most people with Paget's disease of the nipple who don't have a lump will have non-invasive breast cancer.

Diagnosing Paget's disease

You should visit your GP if you notice any changes in the skin of your nipple or areola (the darker area of skin around the nipple).

As Paget’s disease is a form of breast cancer, the sooner it's diagnosed, the better the outcome is likely to be.

Speak to your GP if you develop a lump in your breast. While most breast lumps are not cancerous, it's important you have it checked out.

Read more about:

• the symptoms of breast cancer
• how Paget's disease is diagnosed

Treating Paget's disease

Paget’s disease is treated in the same way as breast cancer. 

Surgery is usually the first line of treatment but unlike other forms of breast cancer, it involves removing breast tissue that includes the nipple and areola.

This may be followed by a combination of:

• chemotherapy – where powerful medication is used to destroy cancerous cells
• radiotherapy – where controlled doses of high-energy radiation are used to destroy cancerous cells
• biological or hormone therapy – which can be used to treat certain types of cancer 

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

Preventing Paget's disease

Modifying certain lifestyle factors, such as reducing alcohol intake and regular exercise, may reduce your risk of developing certain types of cancer, including breast cancer. However, these factors remain controversial.

Screening is also used to help detect breast cancer early. The NHS Breast Screening Programme provides free breast screening every 3 years for all women in the UK who are 47 years of age or over.

Read more about preventing breast cancer.

Risk factors for breast cancer

There are a number of factors that can increase your risk of developing breast cancer. These include:

• age – your risk of developing breast cancer increases as you get older
• family history – if you have a number of close relatives with breast cancer, your chances of also developing the condition are increased
• previously being diagnosed with breast cancer
• previously having a benign breast lump – certain types of benign lump may slightly increase your risk but this is only seen in a small number of women
• being overweight – which is especially significant in post-menopausal women
• alcohol – your risk of developing breast cancer can increase with the amount of alcohol you drink

Information about you

If you have Paget’s disease of the nipple, your clinical team will pass information about you on to the National Congenital Anomaly and Rare Diseases Registration Service (NCARDRS).

This helps scientists look for better ways to prevent and treat this condition. You can opt out of the register at any time.

Find out more about the register.

As Paget’s disease of the nipple is usually a sign of breast cancer, it's very important you speak to your GP if you notice changes to the tissue or skin of your breast.

In particular, you should tell your GP if you notice any changes to:

• the skin of your nipple or areola (the darker area of skin around the nipple)
• your breasts, particularly lumps in your breast 

Paget’s disease of the nipple can sometimes be confused with eczema, a skin condition that also causes red, itchy and dry skin.

Therefore, you should visit your GP for a diagnosis rather than assuming you have eczema. Paget’s disease is a form of breast cancer and the sooner it's diagnosed, the better the outcome is likely to be.

Your GP will examine both breasts, even if you only have a problem with one of them. They may also ask you:

• about your symptoms and how long you have had them
• whether you have a history of eczema, or have eczema anywhere else on your body
• whether you have a personal or family history of breast cancer
• your age and whether you have experienced the menopause (when a woman's monthly periods stop)
• whether you're taking any medication, including hormone replacement therapy (HRT), which is used to treat some of the symptoms of the menopause, or the oral contraceptive pill
• how much alcohol you drink
• how much you weigh and if you have recently gained weight

If your GP thinks you may have breast cancer, they will refer you to a specialist breast clinic for tests. 

Breast clinic

When you visit a breast clinic you will have tests to find out whether you have breast cancer and, if you have, what type of breast cancer.

Staff at the clinic may take photographs of your breasts to record their current appearance and to help identify any further changes that may occur.

Tests carried out at the clinic may include the following:

• an examination of your breast to check for lumps or other abnormalities
• a mammogram (if you are 35 years of age or over)
• an ultrasound scan – which is the first line of investigation used in younger women
• a skin biopsy will be carried out if Paget’s disease is suspected

Mammogram

A mammogram is a simple procedure that uses X-rays to create an image of the inside of your breasts. It can identify early changes in your breast tissue when it may be difficult to feel a lump.

Younger women often have denser breasts than older women, which can make identifying changes more difficult. Therefore, mammograms are not as effective in women under 35 years of age. If you're under 35, your doctor may suggest you have a breast ultrasound instead.

However after a diagnosis of Paget’s disease has been confirmed, mammography will become an important part of pre-surgery assessment.

During a mammogram, the radiographer will position 1 of your breasts on a flat X-ray plate. A second X-ray plate will press down on your breast from above, so that it is temporarily compressed and flattened between the 2 plates.

An X-ray will be taken, which will give a clear image of the inside of your breast. The procedure will then be carried out on your other breast.

Having a mammogram can be slightly uncomfortable or even painful, but the procedure will only take a few minutes. Your doctor will examine the image produced by a mammogram for indications of cancer.

Breast ultrasound

If you are under 35 years of age, a breast ultrasound may be recommended. This is because your breast tissue may be too dense for a mammogram. Your doctor may also suggest a breast ultrasound if they need to find out whether a lump in your breast is solid or contains liquid.

Ultrasound uses high-frequency sound waves to produce an image of the inside of your breasts. An ultrasound probe or sensor will be placed over your breasts to create an image on a screen. The image will show any lumps or abnormalities present in your breasts.

Skin biopsy

A skin biopsy is used to diagnose Paget’s disease. A biopsy is where a small sample of tissue is taken for examination under a microscope and tests to see if it is cancerous.

Further tests

There are a number of other tests that can be used if a diagnosis of breast cancer is confirmed and help determine what types of treatment might be used.

Paget’s disease of the nipple is often associated with other forms of breast cancer.

It's usually treated in the same way as more common types of breast cancer, either by removing the cancerous section of the breast or sometimes by removing the entire breast – a procedure known as a mastectomy.

You can discuss any concerns you have with your oncologist (cancer specialist) who will be able to explain each phase of your treatment.

Surgery

If you're diagnosed with Paget’s disease, surgery is often the first form of treatment you will receive. There are 2 main types of surgery. They are:

• mastectomy – surgery to remove the whole breast, which can be followed by reconstructive surgery to recreate the removed breast
• breast-conserving surgery – surgery to remove the cancerous lump (tumour) in your breast

These types of surgery will also involve removing your nipple and the darker area of skin surrounding it (the areola).

Mastectomy

A mastectomy removes all your breast tissue, including your nipple. You may need to have a mastectomy if:

• the tumour is large or in the centre of your breast
• there is more than one area of breast cancer
• breast-conserving surgery (see below) is not able to provide acceptable results

If lymph nodes (small glands) are removed from your armpit during a mastectomy, the scarring may block the filtering action of the lymph nodes, resulting in a condition called lymphoedema. This is a long-term condition, but can be treated with:

• massage
• compression sleeves – tightly fitting bandages that push excess fluid out of your arm

Lymphoedema can develop months or sometimes years after surgery. See your breast care nurse or GP if you notice any swelling in your arm or hand on the side of your operation.

Breast-conserving surgery

Breast-conserving surgery aims to save as much of your breast as possible while removing the cancer with a rim of healthy tissue.

If you have Paget’s disease, your nipple and areola will be removed. You should be offered reconstructive surgery to improve the appearance of your breast after surgery.

If you have breast-conserving surgery, the amount of breast tissue removed will depend on:

• the size of the tumour in relation to the size of your breast
• whether the tumour is in one place or scattered throughout your breast

Your surgeon will remove an area of healthy breast tissue around the cancer so it can be tested for traces of cancer.

If cancer cells are found in the surrounding tissue, you may need to have more tissue surgically removed from your breast.

After having breast-conserving surgery, it's likely you will need radiation treatment (radiotherapy) to destroy any remaining cancer cells.

Breast reconstruction

If you have a mastectomy, you may be able to have reconstructive surgery to recreate your breast. This can be done by:

• inserting a breast implant
• using tissue from another part of your body to create a new breast

The reconstruction can be carried out at the time of your mastectomy or at a later stage. You should discuss your options fully with your surgeon and breast nurse before making a decision.

For example, it may be possible to have reconstructive surgery after breast-conserving surgery to improve the appearance of your breast and create a nipple.

Creating a nipple

A nipple can be created by:

• having a nipple tattooed onto the skin
• using your own body tissue, such as tissue from your other nipple, although around half of these flatten out and shrink over time
• using a stick-on latex (rubber) nipple, which can be made from a mould of your other nipple so that they are identical; you stick it on every day with glue and it can be removed for washing

Prostheses

If you decide not to have breast reconstruction, you can wear a false breast or breast prosthesis, which are available free on the NHS.

After having a mastectomy, you may have a temporary, fibre-filled prosthesis and a permanent prosthesis made from silicone, which can be replaced every 2 years.

Further treatment

After your surgery, you may need further treatment if you have invasive breast cancer, where the cancerous cells have spread into other tissue in your breast.

If you had non-invasive breast cancer, where the breast cancer cells were contained in one area of your breast, surgery may be all the treatment you need.

Other types of treatment for breast cancer include:

• chemotherapy – cytotoxic medication that prevents cancer cells from dividing and growing is used to destroy cancer cells
• radiotherapy – where controlled doses of high-energy radiation, usually X-rays, are used to destroy cancer cells
• biological therapy – if your breast cancer is HER2 positive, biological therapy, usually a medication called trastuzumab, can be used to treat the cancer by stopping the effects of HER2 and helping your immune system fight off cancer cells
• hormone therapy – if your breast cancer is hormone-receptor positive, hormone therapy can be used to treat the cancer by lowering the levels of hormones in your body or stopping their effects

A number of factors can help reduce your risk of developing breast cancer, including Paget's disease of the nipple.

Diet and lifestyle

Exercising regularly and eating a healthy, balanced diet are known to help prevent many forms of cancer, as well as other serious health conditions, such as heart disease and diabetes.

Studies have looked at the link between breast cancer and diet and, although there are no definite conclusions at the moment, there are benefits for women who maintain a healthy weight, do regular exercise and who have a low intake of saturated fat and alcohol.

It has been suggested that exercising regularly (a minimum of 150 minutes or 2 hours 30 minutes a week) can reduce your risk of developing breast cancer by up to a third.

If you have been through the menopause (when your monthly periods stop), it's particularly important you are not overweight or obese. This is because these conditions cause more oestrogen to be produced, which can increase the risk of breast cancer.

Breastfeeding

Studies have shown that women who breastfeed are statistically less likely to develop breast cancer than those who don't.

The reasons are not fully understood, but it could be because women don't ovulate as regularly while they are breastfeeding and oestrogen levels remain stable.

Medication

In June 2013, the National Institute for Health and Care Excellence (NICE) announced that two medications, called tamoxifen and raloxifene, would be available on the NHS for women who have an increased risk of developing breast cancer.

Either tamoxifen or raloxifene can be used in women who have been through the menopause, but only tamoxifen should be used in women who haven't.

These medications may not be suitable if in the past you have had blood clots or womb cancer, or if you have an increased risk of developing these problems in the future. Women who have already had a mastectomy to remove both breasts won't be offered these medications because their risk of developing breast cancer is so small.

A course of treatment with tamoxifen or raloxifene will usually involve taking a tablet every day for 5 years.

Raloxifene can cause side effects including flu-like symptoms, hot flushes and leg cramps. Side effects of tamoxifen can include hot flushes and sweats, changes to your periods and nausea and vomiting.

Your chances of giving birth to a child with birth defects increases while you're taking tamoxifen, so you'll be advised to stop taking it at least 2 months before trying for a baby. The medication can also increase your risk of blood clots so you should stop taking it 6 weeks before any planned surgery.

Currently, these 2 medications are not licensed for the purpose of reducing the risk of breast cancer in women with an increased risk of developing the condition. However, they can still be used if you understand the benefits and risks and your doctor believes the treatment will be helpful.

Breast screening

Breast screening can pick up breast cancer before it forms a lump. The procedure uses mammograms, where X-rays are taken, to create an image of the inside of your breasts.

The NHS Breast Screening Programme provides free breast screening every 3 years for all women in the UK who are 50 years of age or over. The NHS has recently extended the breast screening age range so that all women who are 47 to 73 years of age will be eligible for breast screening.

NHS breast screening is not usually available for women under 47 years of age. This is because younger women tend to have denser breast tissue which makes mammograms less effective at identifying abnormalities.

Speak to your GP if you are below screening age, worried about changes in your breasts, or have a family history of breast cancer.

Read more information about breast cancer screening.