What is Paget's Disease of the Breast? Symptoms, Causes, & Treatment

Paget's disease of the breast is a rare form of breast cancer that affects the skin of the nipple and areola. This condition accounts for about 1-3% of all breast cancers and typically occurs alongside an underlying breast tumor.

You might notice changes that look like eczema or a rash on your nipple area. While these symptoms can be alarming, understanding what you're dealing with helps you take the right steps toward proper care and treatment.

What is Paget's Disease of the Breast?

Paget's disease of the breast happens when cancer cells called Paget cells spread from an underlying breast tumor to the skin of your nipple and areola. These special cancer cells create distinctive changes in the skin that doctors can identify under a microscope.

This condition almost always occurs with an invasive breast cancer or ductal carcinoma in situ (DCIS) somewhere else in the breast. Think of it as cancer cells traveling from their original location to affect the nipple area specifically.

The disease was named after Sir James Paget, a British surgeon who first described it in 1874. While it's considered rare, early recognition and treatment lead to good outcomes for most people.

What are the Symptoms of Paget's Disease of the Breast?

The symptoms of Paget's disease often develop gradually and can easily be mistaken for common skin conditions. You might first notice what looks like a persistent rash or irritation that doesn't respond to typical treatments.

Here are the main symptoms you should watch for:

• Persistent redness, scaling, or crusting of the nipple and areola
• Itching or burning sensation in the nipple area
• Flaking or peeling skin that may look like eczema
• Thickening of the nipple or areola skin
• Bloody or clear discharge from the nipple
• Changes in nipple shape or inversion
• A lump or thickening in the breast tissue

These symptoms typically affect only one breast and don't improve with moisturizers or over-the-counter treatments. The key difference from regular skin conditions is that Paget's disease symptoms persist and may gradually worsen over time.

Sometimes, you might not feel a lump in your breast even when Paget's disease is present. This happens because the underlying cancer can be small or located deep within the breast tissue where it's harder to detect through touch alone.

What Causes Paget's Disease of the Breast?

Paget's disease develops when cancer cells from an underlying breast tumor travel through the milk ducts to reach the nipple and areola. The exact reason why some breast cancers spread this way while others don't isn't completely understood by medical researchers.

The most widely accepted theory suggests that Paget cells originate from ductal carcinoma in situ or invasive breast cancer elsewhere in the breast. These cancer cells then migrate along the milk ducts until they reach the surface of the nipple, where they cause the characteristic skin changes.

Another theory proposes that Paget cells might develop independently in the nipple area itself. However, this is less likely since nearly all cases of Paget's disease occur alongside another form of breast cancer in the same breast.

Unlike some other types of cancer, Paget's disease isn't directly caused by lifestyle factors, environmental exposures, or infections. It appears to be a specific pattern of how certain breast cancers behave and spread within the breast tissue.

When to See a Doctor for Paget's Disease of the Breast?

You should see your doctor if you notice any persistent changes in your nipple or areola that last longer than a few weeks. While many nipple changes have benign causes, it's important to have them evaluated properly.

Schedule an appointment promptly if you experience any of these warning signs:

• Nipple rash or irritation that doesn't improve with gentle care
• Scaling, crusting, or flaking skin that keeps returning
• Any discharge from the nipple, especially if it's bloody
• Changes in nipple shape or sudden inversion
• Persistent itching or burning that doesn't respond to treatment
• A lump or thickening anywhere in your breast

Don't wait to see if symptoms resolve on their own, especially if they're affecting only one breast. Early detection and treatment of Paget's disease significantly improve outcomes and treatment options.

If you've been treating what you think is eczema or dermatitis on your nipple for several weeks without improvement, it's time to consult with a healthcare provider. They can determine whether you need further evaluation or referral to a specialist.

What are the Risk Factors for Paget's Disease of the Breast?

The risk factors for Paget's disease are similar to those for other types of breast cancer, since this condition almost always occurs alongside an underlying breast tumor. Understanding these factors can help you stay aware of your personal risk level.

Here are the main risk factors to be aware of:

• Age - most commonly affects women over 50, though it can occur at any age
• Gender - women are much more likely to develop this condition than men
• Family history of breast or ovarian cancer
• Personal history of breast cancer or high-risk breast lesions
• Genetic mutations, particularly BRCA1 and BRCA2
• Dense breast tissue
• Previous radiation therapy to the chest area
• Hormone replacement therapy use

Having one or more risk factors doesn't mean you'll develop Paget's disease. Many people with risk factors never develop breast cancer, while others with no known risk factors do. These factors simply help doctors understand patterns and recommend appropriate screening.

It's worth noting that Paget's disease can occasionally occur in younger women and even in men, though this is quite rare. The condition doesn't discriminate based on risk factors alone, which is why paying attention to symptoms is so important for everyone.

What are the Possible Complications of Paget's Disease of the Breast?

When Paget's disease is caught early and treated appropriately, most people have excellent outcomes. However, like other forms of breast cancer, there can be complications if the condition progresses without treatment.

The main complications you should understand include:

• Spread of cancer to nearby lymph nodes
• Invasion of cancer into deeper breast tissues
• Metastasis to other parts of the body
• Development of additional tumors in the same or opposite breast
• Chronic infection of the nipple area due to open wounds
• Permanent changes to breast shape or nipple appearance

The good news is that when Paget's disease is detected early, the underlying cancer is often still contained within the breast tissue or limited to nearby areas. This makes treatment more effective and reduces the risk of serious complications.

Some people worry about the emotional and psychological impact of dealing with breast cancer. These concerns are completely normal and valid. Many treatment centers offer counseling and support services to help you navigate both the physical and emotional aspects of your diagnosis and treatment.

How is Paget's Disease of the Breast Diagnosed?

Diagnosing Paget's disease requires a combination of physical examination, imaging studies, and tissue analysis. Your doctor will start by carefully examining your nipple and breast area, looking for the characteristic changes that suggest this condition.

The diagnostic process typically includes several steps. First, your doctor will perform a clinical breast exam and ask about your symptoms and how long you've had them. They'll want to know if the changes affect one or both breasts and whether you've tried any treatments.

Next, you'll likely need imaging studies to look for underlying breast cancer. A mammogram is usually the first imaging test, followed by breast ultrasound or MRI if needed. These tests help identify any tumors or suspicious areas that might not be felt during the physical exam.

The definitive diagnosis comes from a biopsy of the affected nipple area. During this procedure, your doctor removes a small sample of skin tissue, which is then examined under a microscope by a pathologist. They'll look for the specific Paget cells that confirm the diagnosis.

If Paget's disease is confirmed, additional tests may be needed to determine the extent of the underlying breast cancer. This might include more detailed imaging studies or additional biopsies to help plan the most appropriate treatment approach for your specific situation.

What is the Treatment for Paget's Disease of the Breast?

Treatment for Paget's disease focuses on addressing both the nipple changes and the underlying breast cancer. Your treatment plan will be tailored to your specific situation, including the size and type of the underlying tumor and whether cancer has spread to lymph nodes.

Surgery is typically the main treatment approach. The most common surgical option is a mastectomy, which removes the entire breast, nipple, and areola. This approach ensures complete removal of both the Paget's disease and the underlying cancer.

In some cases, breast-conserving surgery might be possible if the underlying cancer is small and located away from the nipple area. However, this approach requires removal of the nipple and areola, along with the tumor and surrounding tissue. The decision depends on factors like tumor size, location, and your personal preferences.

After surgery, additional treatments may be recommended based on the characteristics of your cancer. These might include:

• Chemotherapy to destroy any remaining cancer cells
• Radiation therapy to reduce the risk of cancer returning
• Hormone therapy if your cancer is hormone-sensitive
• Targeted therapy drugs for specific types of cancer

Your medical team will work with you to develop a comprehensive treatment plan that addresses your cancer while considering your overall health, preferences, and quality of life goals. Treatment decisions are always made collaboratively between you and your healthcare providers.

How to Manage Symptoms at home During Treatment?

While you're going through treatment for Paget's disease, there are gentle ways to care for yourself at home that can help you feel more comfortable. These supportive measures work alongside your medical treatment, not as a replacement for it.

For nipple and skin care, keep the affected area clean and dry using mild, fragrance-free soap. Avoid harsh scrubbing or irritating products that might worsen skin changes. Instead, gently pat the area dry after washing and avoid tight-fitting bras or clothing that might rub against sensitive skin.

Managing discomfort is important for your overall well-being. Over-the-counter pain relievers like acetaminophen or ibuprofen can help with any discomfort, but check with your doctor first to make sure these medications won't interfere with your treatment.

Emotional support is just as important as physical care. Consider joining a support group for people with breast cancer, whether in person or online. Connecting with others who understand what you're going through can provide comfort and practical advice.

Maintain good nutrition and gentle exercise as approved by your medical team. These habits support your body's healing process and can help you feel stronger during treatment. Remember that rest is also important, so listen to your body and don't push yourself too hard.

How Should You Prepare for Your Doctor Appointment?

Preparing for your doctor appointment helps ensure you get the most out of your visit and don't forget important information. Start by writing down all your symptoms, including when they started and how they've changed over time.

Bring a complete list of all medications, vitamins, and supplements you're taking. Include prescription drugs, over-the-counter medications, and any herbal remedies. Your doctor needs this information to understand potential interactions and plan your care safely.

Gather your medical history, including any previous breast problems, biopsies, or cancer diagnoses. If you have family members with breast or ovarian cancer, write down their relationship to you and their ages when diagnosed. This family history information helps your doctor assess your risk factors.

Prepare a list of questions you want to ask. Don't worry about having too many questions - your doctor expects and welcomes them. Consider asking about:

• What tests will be needed to make a diagnosis?
• What are the possible treatment options?
• What are the risks and benefits of each treatment?
• How will treatment affect my daily activities?
• What support resources are available?

Consider bringing a trusted friend or family member to your appointment. They can provide emotional support and help you remember important information discussed during the visit. Having someone there also gives you another person to ask questions if you think of something later.

What's the Key Takeaway About Paget's Disease of the Breast?

Paget's disease of the breast is a rare but treatable form of breast cancer that affects the nipple and areola. While the symptoms might initially look like common skin conditions, persistent changes that don't improve with basic care warrant medical evaluation.

Early detection makes a significant difference in treatment outcomes. If you notice any persistent nipple changes, scaling, discharge, or irritation that lasts more than a few weeks, don't hesitate to see your doctor. What might seem like a minor skin problem could be something that needs prompt attention.

The most important thing to remember is that effective treatments are available, and many people with Paget's disease go on to live full, healthy lives after treatment. While receiving any cancer diagnosis feels overwhelming, you're not alone in this journey.

Stay connected with your healthcare team, ask questions when you need clarification, and don't be afraid to seek emotional support. Taking care of your mental and emotional health is just as important as addressing the physical aspects of your condition.