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 where the the skin of the nipple becomes red, itchy and dry.

Information written and reviewed by Certified Doctors.

Contents

Key Information

What should I do?

If you think you have this condition you should see a doctor within 48 hours.

How is it diagnosed?

Your doctor might suspect Paget’s disease based on your symptoms and physical examination findings. Scans such as ultrasound or a mammogram are recommended to check for any lumps that cannot be detected during the physical examination of the breast. A small sample of skin from the affected area (biopsy) will be taken and sent for analysis in order to diagnose this cancer.

What is the treatment?

If you are diagnosed with Paget’s disease, then the treatment will depend on the extent of spread and your general health.

  • For tumours that are localised to one area of your breast, you might be advised to have a part or your whole breast surgically removed.
  • If the tumour has spread to other areas of your body, your doctor might recommend chemotherapy or radiotherapy.

Introduction

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

It 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).

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, which may be red, dry, sore or scaly. Other possible symptoms include:

  • itchiness or a burning sensation
  • bleeding
  • discharge from the nipple, which may be streaked with blood

However, if itchiness, burning or bleeding occur on their own, it is unlikely you have Paget's disease of the nipple.

Read more about the symptoms of Paget's disease.

Causes of Paget's disease

Paget’s disease is usually a sign of breast cancer in tissue behind the nipple, or other parts of the breast away from the nipple. The breast cancer can either be:

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

In around half of all cases of Paget’s disease, a lump is found in the breast. About 9 out of 10 people with a lump will have invasive breast cancer.

Of those who do not have a lump, 4 out of 10 people will have invasive breast cancer, and the rest will have in situ breast cancer.

Read about the causes of Paget's disease.

Diagnosing Paget's disease

You should visit your doctor 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 is diagnosed, the better the outcome is likely to be.

See your doctor if you develop a lump in your breast. While most breast lumps are not cancerous, it is important you have it checked out.

Read more about 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 is a good chance of recovery. Read more about how Paget's disease is treated.

Preventing Paget's disease

Modifying certain lifestyle factors, such as diet and exercise, may reduce your risk of developing certain types of cancer, including breast cancer.

Screening is also used to help identify women who may have an increased risk of developing breast cancer. The NHS Breast Screening Programme provides free breast screening every three years for all women in the UK who are 50 years of age or over.

Read more about preventing Paget's disease and breast cancer.

Symptoms

Paget’s disease of the nipple or breast affects the skin of the nipple, plus the darker area of skin around the nipple (the areola).

Just one nipple is usually affected, with the skin on and around it becoming red, dry, sore and scaly. The symptoms are often similar in appearance to those of eczema.

You may also experience:

  • itchiness or a burning sensation
  • bleeding
  • discharge from the nipple, which may be streaked with blood

However, if itchiness, burning or bleeding occur on their own, it is unlikely you have Paget's disease of the nipple.

Breast cancer

Paget’s disease is usually found alongside invasive breast cancer. This is breast cancer that could potentially spread.

In about half of all cases of Paget’s disease, a lump develops in the breast, often in tissue behind the nipple.

It is very important to be aware of any lumps or changes in the appearance, feel or shape of your breasts. The sooner a cancerous lump is detected, the better the chance of treating it successfully.

Other signs of breast cancer include:

  • a lump or swelling in either of your armpits
  • a change in the size or shape of one or both breasts
  • dimpling on the skin of your breasts or the nipple becoming inverted
  • bleeding from your nipple

Read more about the symptoms of breast cancer.

Causes

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

The breast cancer can either be:

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

In about half of all cases of Paget’s disease of the nipple, a lump is found in the breast. About 9 out of 10 people with a lump will have invasive breast cancer.

Of those who do not have a lump, 4 out of 10 people will have invasive breast cancer and the rest will have in situ 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
  • being overweight - use the BMI healthy weight calculator to find out whether you are overweight or obese
  • alcohol - your risk of developing breast cancer can increase with the amount of alcohol you drink

Read more about the risk factors for breast cancer.

Diagnosis

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

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

Your doctor 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 are 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 doctor thinks you may have breast cancer, they will refer you to a specialist breast clinic for tests.

Breast clinic

Breast clinics are run by breast surgeons, breast clinicians or specialist nurses.

When you visit a breast clinic you will have tests to determine 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 40 years of age or over)
  • an ultrasound scan 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 40 years of age. If you are under 40, your doctor may suggest you have a breast ultrasound instead (see below).

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

During a mammogram, a radiographer (X-ray specialist) will position one 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 two 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, such as areas of calcium within your breast tissue that show up on the X-ray (calcification).

Breast ultrasound

If you are under 40 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.

MRI scan

An MRI scan of the breast is often used to assess the extent of a person's breast cancer. Results can help determine which surgical procedure should be carried out - for example, whether all of the breast should be removed (a mastectomy), or just the central part of the breast including the nipple and areola.

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 to confirm a diagnosis of breast cancer and help determine what types of treatment might be used. Read more about these alternative tests.

Treatment

Paget’s disease of the nipple is often associated with other forms of breast cancer. It is usually treated in the same way as more common types of breast cancer.

Surgery to remove the entire breast may be recommended for Paget’s disease. This procedure is known as a mastectomy (see below).

Your treatment will be managed by a team of healthcare professionals led by a cancer specialist (oncologist).

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

Surgery

If you are diagnosed with Paget’s disease, surgery is often the first form of treatment you will receive. There are two 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).

The two different types of surgery are discussed in more detail below.

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.

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, which are 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 doctor if you notice any swelling in your arm or hand on the side of your operation.

Read more about mastectomy, including information and advice about recovery and possible complications.

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 (see below).

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 is 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 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:

  • tattooing a nipple on
  • 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.

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

Read more about how breast cancer is treated.

Prevention

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

Diet and exercise

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 looking at the link between diet and breast cancer have found there are benefits for women who maintain a healthy weight, take regular exercise and avoid consuming high amounts 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.

After experiencing the menopause (when your monthly periods stop at around 52 years of age), it is particularly important you are not overweight or obese. Being overweight can cause more of the female hormone, oestrogen, to be produced, which can increase the risk of breast cancer.

Read more about preventing breast cancer.

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.

Content supplied by NHS Choices