What is Paget's Disease?

Paget's Disease of Bone Explained

Bone is an active living tissue that keeps renewing. When old and damaged bones are broken down, they are replaced with new, healthy bones. In Paget's disease, this renewal process is disrupted, and it develops as a chronic bone disorder in which the normal renewal process of bone becomes defective.

Instead of sequentially restoring the bone to a steady state, the body generates large, oversized, and hyperactive bone cells, which in turn form bones too fast. This pattern of rapid and chaotic bone remodelling can produce bones that are weakened, deformed, and more likely to experience problems.

Read ahead to learn more about this condition alongside its types and symptoms.

What are the types of Paget’s disease?

There are 3 main types of Paget’s disease, each affecting different body parts. These include:

1. Paget's Disease of the Bone (Osteitis Deformans)

This is the most common type and is characterised by a chronic disorder of bone remodelling. In a skeletal structure, new bone tissue gradually replaces old bone tissue. In Paget's disease of the bone, this process becomes haphazard and uncontrolled, resulting in the formation of enlarged, deformed, and weak bones.

Affected areas are usually the pelvis, spine, skull, and lower legs. Although its precise cause is still unclear, genetic predispositions and potentially viral infections can be involved. 

2. Paget's Disease of the Breast (Mammary Paget's Disease)

Also referred to as Paget's disease of the nipple, Paget's disease is a rare type associated with breast cancer. It involves the nipple and areola skin, with typical red, scaly, or pruritic lesions.

The chronic changes in the skin are due to malignant Paget cells, which might precede or progress to underlying ductal breast carcinoma. It has overlapping risk factors for other forms of breast cancer and usually needs imaging, biopsy, and oncologic treatment.

3. Extramammary Paget's Disease (EMPD)

Extramammary Paget's Disease (EMPD) is an unusual form of skin cancer that usually occurs in parts of the body with a high concentration of apocrine sweat glands—i.e., the vulva, perianal region, penis, scrotum, perineum, and axillae.

It is defined by malignant Paget cells in the epidermis. EMPD is further classified into 2 categories: primary EMPD, which starts from the skin or apocrine glands, and secondary EMPD, which could be due to underlying cancers, including adenocarcinomas.

What are the symptoms of Paget’s disease?

Paget's disease tends to develop without the presence of notable symptoms, particularly in the initial stages. Most people do not know anything about the condition until it is discovered through standard X-rays or blood tests. When symptoms appear, they involve:

• Bone Pain: It is one of the most common symptoms, frequently experienced in the pelvis, spine, skull, or long bones such as the thigh or shin.
• Joint Pain and Stiffness: This may result when the disease involves bones close to joints, making osteoarthritis more likely to develop.
• Bone Fractures: Pagetic bones are dense but structurally disorganized, making them prone to fractures, but this is distinct from osteoporosis.
• Bone Deformities: Can cause an enlarged head, bowed legs, spinal curvature, or abnormal limb shape, typically in the advanced stage.
• Hearing Loss and Headaches: These can occur if the skull is affected by pressure on nerves or bone architectural changes.
• Compression of Nerves: Paget's disease in the skull or spine can result in nerve impingement, which can cause pain, numbness, or a tingling sensation.
• Increased Heat Over Involved Bones: Due to increased blood supply to the sites of active bone turnover, typically perceived in surface bones such as the shins.
• Hip Pain: Hip pain and stiffness can occur if the femur or pelvis is affected, impairing mobility and weight-bearing capacity.
• Damage to Joint Cartilage: Chronic bone shape changes and joint stress can wear cartilage down and cause secondary arthritis.

Paget's disease of bone can be treated when identified at an early stage and treated accordingly. Early intervention requires recognition of its symptoms, risk factors, and complications. With new therapies and lifestyle modifications, a person with Paget's disease can be healthy and active.

It is also advisable to opt for health insurance to secure your future health and finances during emergencies. At Star Health, our insurance plans offer comprehensive coverage for various diseases. We also provide hassle-free claim settlements with reduced turnaround time across over 14,000 network hospitals in India.

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