215-322-5256Table of Contents
What Is Paget’s Disease?
Paget’s disease is a chronic condition that interferes with your body’s normal bone remodeling process. Essentially, bone remodeling is where new bone tissue gradually replaces old bone tissue. This relationship seems to be essential to ensure normal amounts of calcium in our blood. Paget’s bone disease is most common in the pelvis, skull, spine, and legs.
The probability of Paget’s bone disease rises with age. There are two forms of Paget’s disease of the bone:
- Monostotic: In the monostotic type, one bone site is affected by Paget’s disease.
- Polyostotic: In this type, several sites on the bone are affected by Paget’s disease.
When the disease affects the skull, it is called “Paget’s disease of the skull.”
Symptoms
Some patients with Paget syndrome have no symptoms at all. The disorder is also only identified when x-rays are taken for some unrelated cause or when normal blood work shows elevated alkaline phosphatase levels.
Bone pain is perhaps the most frequent complaint in people with symptoms. This discomfort can be linked to the complications of active Paget’s disease, including:
- Fractures because of delicate or brittle bone
- Bone disfigurement
- Joint arthritis around the bone
- Compression of the adjacent nerves from the swollen bones, resulting in lack of feeling or movement
Paget’s disease may also result in Paget’s sarcoma, a bone cancer. When this happens, signs can include extreme and unrelenting pain in the region caused by Paget’s disease. Only one percent of individuals suffering from Paget’s disease may experience Paget’s sarcoma, and their age group falls above 70 years. This form of malignant bone tumor is very active, with a bad prognosis. None of the prescription therapies for Paget’s disease is considered to minimize the chance of developing Paget’s sarcoma.
Causes and Risk Factors
The causes of Paget’s disease of bone remain undetermined. However, it is considered a genetic condition by various studies. However, several risk factors may increase the probability of developing Paget’s disease of the bone. They are as follows:
- Age: Individuals aged 40 and above are most susceptible to Paget syndrome.
- Gender: Men are more likely to develop the disease.
- Nationality: Paget’s disease is common in nations such as England, Greece, Scotland, central Europe, and countries with immigrant Europeans.
- Genetics: A family history is another likely risk factor for the disease.
Diagnosis
Paget’s disease diagnosis is commonly made by three examinations – X-rays, bone scans, and blood tests. The disorder is also often discovered by mistake when one of these experiments is performed for another cause.
X-Ray
In X-ray images, the bones afflicted by Paget’s disease have a specific look that varies from most bones. The doctor can then use X-rays, either alone or with a blood test or a bone scan, to determine whether or not a person has Paget’s bone disease.
Blood Test
Often, blood samples’ findings first alarm doctors to the risk of a person having Paget’s disease. When blood contains a greater than normal amount of a chemical called serum alkaline phosphatase (SAP), that is an indication that the condition may be present. SAP is a type of enzyme made up of bone cells that are overproduced by the pagetic bone.
A small rise in SAP, up to twice the normal amount, may suggest Paget’s disease or another illness, such as liver disease or a bone fracture in the healing process. However, an SAP level greater than twice the average level strongly indicates Paget’s disease, particularly if the person’s serum calcium, phosphorus, and kidney function are normal.
Bone Scan
A bone scan is an examination that helps to determine which bones have been damaged by Paget’s disease. The method is also a valuable means of assessing the degree and activity of the disease. In a bone scan, a healthy quantity of radioactive material is pumped into the human’s vein. The drug circulates through the bloodstream and “highlights” the skeleton sites where Paget’s disease may be present. If the scan shows that a person has an illness, the infected regions are usually x-rayed to validate the diagnosis.
Treatment
A cure for this disease is not available. The treatment of Paget’s disease includes non-surgical treatment such as nonsteroidal anti-inflammatory drugs (NSAIDs), bisphosphonate medications, and surgical treatments – such as internal fixation, osteotomy, and total joint replacement.
What Is Paget’s Disease of the Breast?
Also known as Paget’s disease of the nipple, this condition is associated with breast cancer. It induces eczema-like changes in the skin of the nipple and the darker skin around the nipple (areola). That is typically a symptom of breast cancer in the tissue below the nipple. About 1 to 4 percent of women with breast cancer have Paget’s nipple disease. Men can also be affected, although this is much rarer.
Symptoms
Paget’s disease of the breast always begins in the nipple and may spread to the areola. It looks to be a red, scaly rash on the skin of the nipple and areola. The skin rash often becomes sore and swollen and can be scratchy or cause a burning feeling. Often the nipple could be ulcerated. The texture of the rash is sometimes comparable to other skin conditions, including eczema or psoriasis. When you scratch it, or whether it’s left unchecked, this can bleed, ulcerate, or scab over.
Causes
The precise cause of Paget’s disease of the breast is not known. One hypothesis is that a female already has breast cancer, and certain cancer cells move through the milk ducts to the surface of the nipple. Another possible reason is that the nipple skin cells naturally turn into cancer cells.
Risk Factors
Risk factors of Paget’s disease of the breast are similar to those in other forms of breast cancer, such as increasing age, previous breast cancer history, family history of breast cancer, obesity, hormone replacement therapy, and high alcohol intake levels.
Diagnosis
Paget’s breast disease is diagnosed based on a detailed clinical evaluation, recognition of distinctive anatomical findings, a comprehensive patient history, and several specialized examinations. The condition can be associated with other skin disorders, such as eczema, which may cause a delay in diagnosis.
Tests that can be used to detect Paget’s breast disease better include:
- Blood tests
- Surgical removal (biopsy) and microscopic analysis of infected breast tissue
- Mammography or other advanced breast screening
- Measurement of the discharge of nipples of cancer cells (cytologic smear)
Treatment
The treatment for Paget’s disease of the breast includes removal of the complete breast or through breast-conserving surgery along with whole-breast radiation therapy. If BCS is done, the entire area of the nipple and areola must also be removed. If invasive cancer is detected, the lymph nodes under the arm will be checked for cancer.
If you need close care for Paget’s disease symptoms, reach out to All American Hospice. Our caregivers are well-trained in providing compassion, care, and a helping hand to make living with such diseases more comfortable. Give us a call today!
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