Paget's disease of the breast

Definition-Paget's (PAJ-uts) disease of the breast is a rare form of breast cancer, accounting for less than 5 percent of all breast cancers. Paget's disease of the breast starts in the breast ducts and extends to the skin of the nipple and to the dark circle of skin (areola) around the nipple. Paget's disease of the breast isn't related to Paget's disease of the bone, a metabolic bone disease.
Paget's disease of the breast occurs most often in women over age 50. Most women with Paget's disease of the breast have underlying infiltrating ductal breast cancer. Only in rare cases is the cancer confined to the nipple itself.

Paget’s disease of the breast is an eczema-like change in the skin of the nipple, and 9 out of 10 women who have this have an underlying breast cancer. The underlying breast cancer may be an invasive breast cancer or ductal carcinoma in situ (DCIS). In DCIS, the cancer cells are completely contained within the milk ducts.
Around half of the women who have Paget’s disease will have a breast lump that can be felt at the time it is diagnosed.
Paget’s disease occurs in about 1–2 out of every 100 women with breast cancer. It usually occurs in women in their 50s, but can occur at an younger or older age.
It can affect men, but this is extremely rare. It usually first appears as a scaly, red rash affecting the nipple and sometimes the dark area of skin surrounding the nipple (the areola). The rash always affects the nipple first, and may then affect the areola. It does not go away and may become sore. The area may bleed slightly.

Treat your Paget's Disease

Paget’s disease causes bones to grow too large and weak. You can have Paget’s disease in any bones in your body, but most people have it in their spine, pelvis, skull, or leg bones. The disease may affect only one bone or several bones, but it does not affect the entire skeleton. Bones with Paget’s disease may break more easily, and the disease can lead to other health problems.

Who Gets Paget’s Disease?

About one million people in the United States have Paget’s disease. The disease is more common in older people and those of Northern European descent. Men are more likely than women to have the disease.

What Causes Paget’s Disease?

Doctors are not sure what causes Paget’s disease. They think that a virus may cause it in some cases. It also tends to run in families. Your doctor might suggest that your brothers, sisters, and children have blood tests every 2 or 3 years starting at the age of 40 to check for the disease.

What Are the Symptoms of Paget's Disease?

Many people do not know they have Paget's disease because they have only mild symptoms. For others, signs and symptoms can include:

• Pain
• Enlarged bones
• Broken bones
• Damaged cartilage in joints.

If you have Paget's disease in your leg bones, you may also have bowed legs. Your spine might curve if the disease is in the bones of the spine.

People with Paget's disease in the bones of the skull sometimes have:

• Headaches
• Hearing loss
• Large head size

Symptoms get worse slowly, and the disease does not spread to other bones.

How Is Paget's Disease Diagnosed?

Most often, Paget's disease is diagnosed with x rays. Your doctor may also order:

• An alkaline phosphatase blood test. People with Paget's disease have too much of this enzyme in their blood.
• A bone scan. A bone scan can show which bones the disease has affected.

Early diagnosis and treatment can prevent some symptoms from getting worse.

What Other Medical Problems Might Occur?

Paget's disease may lead to other medical problems, such as:

• Arthritis. The long bones in your leg may bow and press on nearby joints. Enlarged bone may cause more wear and tear on joints. In these cases, joint pain may be due to both Paget's disease and arthritis.
• Hearing loss. If you have Paget's disease in your skull or the bone surrounding your inner ear, you may lose some hearing. Treatment may slow or stop the hearing loss. Hearing aids may also help.
• Heart disease. In severe Paget's disease, the heart has to work harder to pump blood to affected bones.
• Kidney stones. Kidney stones are more common in people with Paget's disease.
• Nervous system problems. Bone with Paget's disease can press on the brain, spinal cord, or nerves. This can slow blood flow to the brain and spinal cord and cause pain.
• Bone cancer. Rarely, people with Paget's disease get cancer of the bone. This can cause sudden or increased bone pain.
• Loose teeth. If you have the disease in bones of your face, your teeth may become loose. This makes it hard to chew food.
• Vision loss. Rarely, people with Paget's disease in the skull may lose some vision.

Do I Need to See a Special Doctor? What Kinds of Doctors Specialize in This Disease?

Paget's disease can affect many parts of the body. You may need to see one or more types of doctors, such as:

• Endocrinologists (doctors who treat metabolic bone diseases like Paget's disease)
• Rheumatologists (doctors who treat arthritis and other joint problems)
• Orthopaedic surgeons (doctors who treat bone problems)
• Neurologists (doctors who treat brain and nerve problems)
• Otolaryngologists (doctors who treat ear, nose, and throat problems).

How Is Paget's Disease Treated?

Paget's disease is treated with medicine and sometimes surgery. A good diet and exercise are also important.

Medicine

Two main types of medicines are approved to treat Paget's disease.

• Bisphosphonates. These medicines help relieve pain and keep the disease from getting worse.
• Calcitonin. This is a hormone made by the thyroid gland. It may be used for certain patients but it does not work as well as bisphosphonates and is not used as often.

Surgery

Surgery is sometimes needed to treat broken bones, malformed bones, or severe arthritis.

• Broken bones. Surgery may be needed to set a broken bone.
• Malformed bones. Surgery to straighten bones may reduce the pain in joints such as the knee.
• Severe arthritis. People with severe arthritis are treated with medicine and physical therapy. If these do not work well, a hip or knee may need to be replaced.

Diet

People with Paget's disease do not need a special diet. But, to maintain strong bones, you should get 1,200 mg of calcium and at least 400 IU of vitamin D every day. After age 70, you should take 600 IU of vitamin D each day. If you have had kidney stones, talk with your doctor about how much calcium and vitamin D to take.