Paget’s Disease of the Breast

April 10, 2025

This page was reviewed under our medical and editorial policy by Nikhila Kethireddy, M.D., assistant clinical professor, and Susan Yost, Ph.D., staff scientist, Department of Medical Oncology & Therapeutics Research, City of Hope^® Cancer Center Duarte

Paget’s disease of the breast (PDB) is a rare type of breast cancer that affects the skin of the nipple and sometimes the areola, the darker area around the nipple. It was first identified by Sir James Paget in 1874, who initially noticed the condition in women who had long-term nipple lesions. Eventually, they were found to have breast cancer.

Several conditions that affect other areas of the body were also named after Paget; however, those are not related to breast cancer or Paget’s disease of the breast. Because PDB is so uncommon, many people might not know about it until they or someone they love is diagnosed.

What Is Paget’s Disease of the Breast?

Paget’s disease of the breast is a rare form of breast cancer. While it may start on the skin’s surface, it is usually linked to other tumors deeper in the same breast. These tumors are typically either ductal carcinoma in situ (noninvasive cancer in which abnormal cells develop within the milk ducts of the breast, but haven’t spread to the surrounding tissue) or invasive breast cancer, which means cancer cells have spread beyond the milk ducts.

One of the key signs of PDB is the presence of Paget cells. These cancerous cells are found in the top layer of the nipple and areola skin. Under a microscope, Paget cells appear larger and rounder than normal cells, and they may show up as individual cells or in small clusters.

Paget’s disease of the breast occurs in both men and women, though it is much more common in women. It is estimated that about 1% to 4% of all breast cancer cases involve Paget’s disease.

Is It an Aggressive Form of Cancer?

Paget’s disease of the breast is not generally considered an aggressive form of cancer on its own. However, in most cases, it is found alongside other types of breast cancer, like ductal carcinoma in situ or invasive ductal carcinoma. Invasive breast cancers may spread beyond the milk ducts into nearby breast tissue. Once the cancer has reached this stage, it may also spread to other body parts through the lymphatic system or bloodstream.

Paget’s Disease of the Breast Symptoms

In PDB, the nipple and areola often look red, crusty or scaly, and the skin may be irritated. Some people notice fluid coming from the nipple, which could be bloody or yellowish. The nipple itself might look flat or even inverted. Many patients report itching or a burning sensation in the affected area. Symptoms of Paget’s disease typically only affect one breast.

What Does Paget’s Disease of the Breast Look Like?

The symptoms of Paget’s disease of the breast are easy to mistake for less serious skin conditions like eczema or dermatitis. Since this type of breast cancer rash may resemble other skin issues, doctors may first try to treat it as a skin condition. If the area does not improve with treatment, a breast biopsy might be recommended to check for cancer cells.

What Causes Paget’s Disease of the Breast?

The exact cause of Paget’s disease of the breast is not fully understood, but it is widely believed to be linked to an underlying breast cancer. The theory is that cancerous cells from the ducts of the breast move through the milk ducts to the nipple and surrounding skin.

No specific risk factors are unique to Paget’s disease of the breast, but it shares common risk factors with other types of breast cancer. These include:

• Age over 50
• Dense breast tissue detected on a mammogram
• Personal history of breast issues, such as cancer or abnormal cells
• Inherited gene mutations like BRCA1 and BRCA2
• Family history of breast or ovarian cancer, or both
• Certain ethnic backgrounds with higher breast cancer risk
• Previous radiation exposure, especially to the chest
• Hormone replacement therapy (estrogen) after menopause
• High alcohol consumption

While these factors may increase the risk, not everyone with Paget’s disease has them. Conversely, some people with these risk factors do not develop the condition.

Diagnosing Paget’s Disease of the Breast

Doctors diagnose Paget’s disease of the breast by performing a nipple biopsy. Several types of biopsies might be used.

A surface biopsy: A glass slide or tool gently scrapes cells from the skin’s surface.

A shave biopsy: A razor-like tool that removes the top layer of skin.

A punch biopsy: A circular tool to cut out a small, disk-shaped piece of tissue.

A wedge biopsy: A scalpel is used to remove a small wedge of tissue.

Since most people with Paget’s disease of the breast also have tumors inside the breast, the doctor will also perform a clinical breast exam to check for breast lumps or other changes. About half of the patients with Paget’s disease have a lump that may be felt during this exam. To further investigate, the doctor may order imaging tests like a diagnostic mammogram, a breast ultrasound or magnetic resonance imaging (breast MRI) scan to look for any underlying tumors. These additional tests help confirm the diagnosis and guide treatment.

Treating Paget’s Disease of the Breast

Paget’s disease is classified using the same TNM (tumor, node, metastasis) staging system as other breast cancers. This system considers characteristics of the original tumor, including whether it has spread to lymph nodes nearby and whether it has spread to other parts of the body. The presence of Paget cells does not change the staging of any underlying cancer. It is the status of the underlying cancer that plays the biggest role in the overall treatment plan.

Treatment of Paget’s disease of the breast typically involves surgery. Traditionally, this meant a mastectomy (removal of the entire breast) with or without the removal of lymph nodes under the arm. This was because most patients with Paget’s disease also had tumors elsewhere in the same breast. Sometimes these were far from the nipple and areola and were unable to be addressed with breast cancer surgery focused only on those areas.

However, recent studies have shown that breast-conserving surgery with removal of the nipple and areola followed by whole-breast radiation therapy may be an option for patients whose mammogram shows no lump or detectable tumor. This approach allows patients to keep more of their breast tissue while appropriately treating the disease.

For patients with a detectable tumor, a mastectomy may still be necessary. In these cases, doctors typically recommend a biopsy of the nearest lymph node (sentinel biopsy) to see if the cancer has spread to the lymph nodes. If cancer is found in the nearest node, more extensive lymph node surgery may be needed.

The care team may recommend additional treatments like chemotherapy or hormone therapy depending on the cancer’s stage and features, such as whether hormone receptors or HER2 proteins are present.

Paget’s Disease of the Breast Survival Rate

The prognosis for Paget’s disease of the breast depends on how the disease presents and whether invasive cancer or lymph node involvement is present. When Paget’s disease is found alongside a palpable breast lump, it is usually a sign of more advanced cancer. If no lump is present, the prognosis tends to be more favorable.

Survival rates are based on patients in the past and treatments that may have changed. They are population estimates, and they do not predict what will happen for an individual patient. With advances in detection and treatment, survival rates today may be different.

Women in the United States diagnosed with Paget’s disease of the breast between 1988 and 2001 had a five-year relative survival rate of 82.6%. This is slightly lower than the 87.1% survival rate for all breast cancer cases.

For women with Paget’s disease and invasive cancer in the same breast, survival rates decrease as the stage of the cancer advances. For example, the five-year relative survival rate is 95.8% for Stage 1; 77.7% for Stage 2; 46.3% for Stage 3; and 14.3% for Stage 4.

There are few clinical trials available for patients with Paget’s disease of the breast because it is so rare. However, some patients with Paget’s disease may be eligible to enroll in some general breast cancer trials. Patients should check with their care team for more information.