There are several types of Paget’s disease. Each one is named after Sir James Paget, an English surgeon who identified the conditions in the late 1800s.
The conditions are categorized based on the body parts they affect. They include Paget’s disease of the breast (nipple and areola), extramammary Paget’s disease (vulva and other areas), and Paget’s disease of the bone.
The treatment options vary depending on the type of Paget’s disease.
In this article, we’ll discuss how each one is diagnosed and treated. We’ll also cover the possible risks and side effects of Paget’s disease treatment.
There are three main types of Paget’s disease. They affect different parts of the body and therefore require different treatments.
Paget’s disease of the breast
Paget’s disease of the breast is associated with breast cancer. It’s also known as mammary Paget’s disease and Paget’s disease of the nipple.
The condition affects the skin of the breast, usually the nipple and areola. It involves chronic lesions caused by malignant cells called Paget cells. These cells can turn into breast cancer later on.
Paget’s disease of the breast has similar risk factors to other breast cancers.
Extramammary Paget’s disease
Extramammary Paget’s disease is a type of carcinoma or cancer that affects epithelial tissue. It develops in certain areas with many apocrine sweat glands. These glands release fat-rich sweat with a distinct odor.
Typically, extramammary Paget’s disease affects the:
- vulva (most common)
- perianal area
- axilla (armpit)
Extramammary Paget’s disease is also characterized by lesions caused by Paget cells.
Primary extramammary Paget’s disease originates in the skin and sweat glands. Secondary extramammary Paget’s disease is typically due to adenocarcinoma somewhere else in the body.
Paget’s disease of the bone
Paget’s disease of the bone is also known as osteitis deformans. It’s a skeletal disorder that affects the bone’s natural remodeling process.
In healthy bones, old bone is replaced with new bone. In Paget’s disease of the bone, the new bone is weak and misshapen.
The exact cause of the condition is unknown. In some cases, an underlying virus may be involved.
In rare cases, the condition can progress to Paget’s sarcoma, a type of bone cancer.
In all types of Paget’s disease, your doctor will begin with a physical examination. They’ll check for skin lesions and, in Paget’s disease of the bone, swelling, and deformities.
Your doctor will also ask questions about your medical history. This will help determine your risk.
Other diagnostic tests will depend on the type of Paget’s disease:
- Paget’s disease of the breast: This type is diagnosed using a biopsy, which involves removing and testing a piece of the skin lesion. Your doctor might also use imaging tests, like a mammogram or ultrasound.
- Extramammary Paget’s disease: Depending on the area, a medical professional will examine the skin lesions using imaging tests or a thin camera. They might also perform a biopsy to check the cells.
- Paget’s disease of the bone: This condition is diagnosed using X-rays, lab tests, and bone scans. A biopsy might be used to rule out other diseases.
For Paget’s disease of the breast, treatments may include:
- breast-conserving surgery
- radiation therapy
- removal of lymph nodes
Extramammary Paget’s disease can be treated using:
- surgical removal of skin lesions
- radiation therapy
- photodynamic therapy
Treatment for this condition includes:
- nonsteroidal anti-inflammatory drugs (NSAIDs)
- assistive devices (cane or brace)
- bisphosphonate medications
- surgery (internal fixation, osteotomy, or total joint replacement)
- radiation therapy
The possible complications depend on the type of Paget’s disease. Side effects may include:
- Lymphedema: The removal of lymph nodes in Paget’s disease of the breast can lead to lymphedema, which is fluid buildup in the limbs.
- Early menopause or infertility: These side effects may be due to chemotherapy or radiation, which might be used for all Paget’s disease types.
- Other cancers: Similarly, chemotherapy and radiation therapy can increase the risk of other cancers.
- Cosmetic skin damage: If a Paget’s skin lesion is removed, it can cause cosmetic skin damage.
- Skin irritation: Topical medications, which are used to treat extramammary Paget’s disease, might irritate the skin.
- Pain: This is a possible side effect of bisphosphonates, which are used to treat Paget’s disease of the bone. This includes muscle cramps and abdominal pain.
Since there are multiple types of Paget’s disease, the cost of treatment can vary widely.
In general, your health insurance provider should cover some or all of the cost of treatment. That’s because it’s considered to be medically necessary. However, some insurers might have restrictions or requirements for certain treatments.
The best way to know how much coverage you’ll receive is to call your health insurance provider. You’ll always want to check on deductibles, coinsurance, and out-of-pocket costs.
To get an idea of the cost, here are the typical fees for various treatments:
According to the CostHelper site, costs for a mastectomy, chemotherapy, and radiation may range in cost as follows:
- Mastectomy: $15,000 to $55,000
- Chemotherapy: $30,000 or more
- Radiation therapy: $10,000 to $50,000
As for other treatment options, the below information on cost may also be helpful:
- Oral bisphosphonates: $123 to $1,874 per year (according to this 2020 cost analysis)
The cost may be higher if you develop complications after a procedure.
Treatment for Paget’s disease depends on the type. Paget’s disease of the breast is typically treated with a mastectomy, chemotherapy, or radiation. Extramammary Paget’s disease, which commonly affects the vulva, requires the removal of skin lesions. Chemotherapy or radiation might also be used.
Paget’s disease of the bone is treated with NSAIDs, bisphosphonates, assistive devices, or surgery to correct bone issues. If it progresses to bone cancer, chemotherapy or radiation therapy might be used.
If you’re diagnosed with Paget’s disease, your healthcare professional will determine the best treatments for your condition.