What Is Paget’s Disease Of The Breast

Introduction 

Paget’s disease of the breast is a rare form of cancer affecting the skin of the nipple and the areola (dark-coloured area of skin around the nipple). It is also known as Paget’s disease of the nipple and affects 1 to 2 out of every 100 women with breast cancer. It appears as a red, itchy and scaly rash on the skin and sometimes causes a burning sensation. Sir James Paget discovered Paget’s disease of the breast in 1874. He identified female patients with ulcerative lesions around the nipple who all later developed breast cancer. 

Paget’s disease of the breast is associated with ductal carcinoma in situ (early-stage breast cancer) or invasive ductal carcinoma (cancer has spread to other parts of the breast tissue).¹ It is less common for people who have Paget’s disease of the breast with no underlying cancer. Paget’s disease of the breast commonly affects post-menopausal women but can affect younger females.²

This condition is often misdiagnosed as the symptoms are similar to other common skin rashes that a person may experience in their life. This article aims to clear any misconceptions. It is important to become familiar with the general symptoms of breast cancer.

Signs and symptoms of paget’s disease of the breast

Paget’s disease of the breast usually affects only one breast. The first symptoms you’ll experience always begin in the nipple and may extend to the areola. It can be uncomfortable, itchy and painful. You may be tempted to scratch the affected area. If you scratch it or leave it untreated, it can bleed and become ulcerated (develops into a painful sore). Approximately 50% of people assigned female at birth who are diagnosed with Paget’s disease have a lump in the breast. Other symptoms include: 

• Discharge or bleeding from the nipple 
• Inflammation around the nipple and areola 
• Burning sensation 
• Nipple turning inwards
• Skin thickening of the nipple
• Crust on the nipple 

It’s likely not to be Paget’s disease of the breast if you’re only experiencing itchiness or bleeding and the nipple looks normal. Regardless, you should still visit a doctor to have it examined. 

Paget’s disease of the breast and underlying breast cancer

9 out of 10 cases of Paget’s disease of the breast will have breast cancer. This could be either ductal carcinoma in situ (DCIS) or invasive breast cancer.²

DCIS is an early-stage breast cancer where the cancer cells only reside in the ducts inside the breast tissue. If left untreated, it could become an invasive cancer. This means the cancer cells have broken out of the ducts and spread to the surrounding breast tissue, lymph nodes or other parts of the body.³

Causes of paget’s disease of the breast

The causes of Paget’s disease of the breast are unknown. People assigned female at birth are more likely to develop breast cancer but typically not Paget’s disease. As with most breast cancers, there’s no specific cause that develops into a certain type of breast cancer. There are risk factors that can increase your chances of developing breast cancer, such as:

• A family history of breast cancer 
• Being overweight 
• Excessive smoking and drinking
• Age - breast cancer is common amongst those over the age of 50
• You were diagnosed with breast cancer previously
• Hormonal factors
  • Taking the contraceptive pill (the risk reduces if you stop taking it)
  • Not having had children 
  • Taking hormone replacement therapy for more than 5 years, especially taking combined hormone replacement therapy 

Having one or more risk factors does not mean you will have breast cancer, and having no risk factors does not mean you will get it. 

Diagnosis

Clinical examination

If you notice any changes in the appearance of the skin around your nipple or you develop a lump in your breast, you should see your GP for a thorough evaluation of your condition. It is expected a thorough history is taken, such as the length of time the lesions have appeared and the associated symptoms. In Paget’s disease of the breast it typically affects one breast in comparison to eczema, which typically affects both breasts.¹ Your GP will also ask about risk factors for breast cancer like family history, habits and medications.¹

Biopsy and histopathological analysis

A diagnosis of Paget’s disease can be made through a punch biopsy.² A punch biopsy makes a small hole in the skin to examine the full thickness of the skin for cancer under a microscope. Before performing a biopsy, you may be given a local anaesthetic to numb the area. You’ll usually experience some mild pain as the anaesthetic is being injected, but this is normal. The anaesthetic helps reduce the pain as much as possible as the tissue is being removed. 

If the results indicate Paget’s disease, then a doctor will send you for a mammogram, an x-ray used to examine any signs of breast cancer. During a mammography, a radiographer will place one breast at a time on an X-ray plate. Another x-ray plate from above will press your breasts firmly for a few minutes. This may cause discomfort or even pain, which will only last for a moment. Speak to your mammographer if you’re experiencing any discomfort, as they can stop at any time.⁴

Treatment approaches

The first line of treatment for Paget’s disease of the breast is surgery. Your doctor will discuss the best options for you depending on whether the cancer has spread beyond the breast ducts and into the surrounding tissue. You may be recommended a mastectomy (removal of the entire breast) if the disease has affected a large area of the breast or an excision of just the affected area. A breast reconstruction surgery may be recommended to reconstruct a better appearance of your breast.

Adjuvant therapies

You may need additional treatment if you have Paget’s disease of the breast and breast cancer. Typical treatments for breast cancer include:

• Chemotherapy – Anti-cancer medicine is either injected intravenously (through the vein) or taken in tablet form and destroys cancer cells 
• Radiation therapy – A high dose of radiation used to kill cancer cells
• Hormonal therapy – Lowers or blocks the amount of hormones responsible for cancer progression. 

Recurrence and follow-up

After a series of treatments for Paget’s disease of the breast, you will have regular checkups with your doctor to discuss how you are doing and if you require any additional support. The number of checkups you’ll have depends on how you’re feeling and the types of treatment you’re receiving or had. Finishing treatments for Paget’s disease is an extraordinary feeling, but some may experience anxiety, as visiting a hospital can bring back some anxiety you may have had during your treatments. 

If you’re experiencing any worries or overwhelming feelings, it’s important to speak to someone who you’re comfortable with about how you’re feeling. Many find it helpful to seek support groups or counselling sessions at any point, whether that’s during or after treatment. 

Impact on quality of life

Your mental health may be impacted if you’re diagnosed with Paget’s disease. You may experience feelings of sadness, anger and even uncertainty. No matter what you’re feeling, there’s help available. There are a number of different organisations there to support you and help you cope with cancer and the treatments. Support and advice can be found through organisations such as Cancer Research UK, the Pink Ribbon Foundation and Macmillan Cancer Support to name but a few.

Summary

Paget’s disease of the breast is typically associated with ductal carcinoma in situ or invasive breast cancer. Their symptoms appear similar to eczema or dermatitis, where the nipple and areola are itchy, painful, ulcerated and sometimes inverted. Paget’s disease of the breast usually affects one breast, a feature that can be distinguishable between eczema and dermatitis. It’s important to speak to your doctor as soon as possible if you notice any changes in the skin around the nipple. A self-examination of the breast is encouraged by feeling for lumps in your breasts. The earlier you recognise the signs and symptoms, the sooner it can be diagnosed and the better the outcome will be.