Paget's disease

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Overview

A disease that disrupts the replacement of old bone tissue with new bone tissue.

Paget's disease of bone most commonly occurs in the pelvis, skull, spine and legs. Risk factors include increasing age and a family history of the condition. Over time, affected bones may become fragile and misshapen.

This condition can be symptomless for a long period of time. When symptoms do occur, they may include bone deformities, broken bones and pain in the affected area.

Treatment involves medication that reduces the breakdown of bone.

Symptoms

Flaky or scaly skin on your nipple.

Crusty, oozing or hardened skin resembling eczema on the nipple, areola or both.

Itching.

Redness.

A tingling or burning sensation.

Straw-colored or bloody nipple discharge.

A flattened or turned-in (inverted) nipple.

Causes

Scientists suspect a combination of environmental and genetic factors contribute to the disease. Several genes appear to be linked to getting the disease. Some scientists believe Paget's disease of bone is related to a viral infection in your bone cells, but this theory is controversial.

Risk factors

Factors that can increase your risk of Paget's disease of bone include:


Age. People older than 50 are most likely to develop the disease.

Sex. Men are more commonly affected than are women.

National origin. Paget's disease of bone is more common in England, Scotland, central Europe and Greece — as well as countries settled by European immigrants. It's uncommon in Scandinavia and Asia.

Family history. If you have a relative who has Paget's disease of bone, you're more likely to develop the condition.

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Complications

Paget's disease of bone can sometimes lead to further problems.


Broken bones

Bones affected by Paget's disease of bone tend to be more fragile than normal bone and are more likely to break (fracture) – even after a relatively minor injury.


Signs of a fracture include:


sudden, severe pain

swelling or tenderness around the injured area

bleeding, if the bone has damaged the tissue and skin 

It's not clear whether bisphosphonate medication helps prevent or treat fractures. If you break a bone, you may need surgery to align the broken bones so that they heal correctly.


Bone deformities

It's common for Paget's disease of bone to affect the appearance of affected bones.


For example, the condition can cause:


enlarged or misshapen bones

the legs to curve outwards (bow legs)

the spine to curve to the sides (scoliosis)

the upper back to become very hunched over (kyphosis)

As with fractures, it's not clear whether bisphosphonates can help prevent deformities. If they do occur, surgery may be carried out to correct them.


Hearing loss

If Paget's disease of bone affects the skull, there's a significant risk that it could lead to permanent hearing loss and possibly total deafness.


This can occur as a result of damage to the bones or nerves that connect the ears to the brain.


It's not known whether treating Paget's disease of bone helps reduce the risk of losing your hearing, but treatment is usually recommended if the condition is affecting your skull.


Too much calcium in the blood

In rare cases, the increased cycle of bone renewal in Paget's disease of bone can result in calcium building up in the blood. This is known as hypercalcaemia.


It usually only occurs in people who have been confined to bed after an operation or a fracture.


Symptoms of hypercalcaemia can include:


extreme tiredness

depression

drowsiness

constipation

new or worsening bone pain

Hypercalcaemia can be treated using medicines to lower blood calcium levels and slow down bone regeneration.


Heart failure

The new bone that forms in people with Paget's disease of bone often contains more blood vessels than normal bone, which can mean the heart has to work harder to pump blood around the body.


Very occasionally, the heart may not be able to pump enough blood around the body. This is known as heart failure.


Symptoms of heart failure can include:


shortness of breath

extreme tiredness and weakness

swelling in the legs, ankles and feet (oedema)

Heart failure can be treated with medicine and in some cases heart surgery. Read more about how heart failure is treated.


Bone cancer

Bone cancer is a rare complication of Paget's disease of bone. It's estimated to affect between 1 in 100 and 1 in 1,000 people with the condition.


Symptoms of bone cancer are similar to those of Paget's disease of bone. They can include:


bone pain

swelling around the affected bone

a lump in the affected bone

Prevention

Unfortunately, there’s nothing you can do to prevent Paget’s disease of the bone. There are some things that are considered risk factors for developing Paget’s disease of the bone, including:


Your age: People over the age of 50 are more likely to develop Paget’s disease of the bone.

Your national origin: Paget’s disease of the bone is more common in European populations, including those living in England, Italy and Spain. It’s rare among Scandinavians and non-European immigrants living in Europe.

Your sex: People assigned male at birth are slightly more likely to develop Paget’s disease of the bone than people assigned female at birth.

Your family history: Paget’s disease of the bone can sometimes run in families. If you have a family member who has the disease, you may be more likely to develop it.