Scleroderma

Scleroderma [skleer-uh-DUR-muh], which means “hard skin,” is a chronic (long-term) disease that is most often diagnosed in women between the ages of 30-50. It is a degenerative [dee-JEN-uhr-it-iv] disease, meaning that it grows worse over time. Scleroderma is rare in children and is the result of an autoimmune response in which the body attacks its own healthy tissue. This can affect the joints, skin, organs, and even blood vessels. There are two primary types of scleroderma.

• Localized. This is when the condition is confined to one area of your child’s body. When there are patches of rough skin from scleroderma it is called morphea [mor-FEE-a]. These patches may occur anywhere on the arms, legs, torso or head. A less common type of localized scleroderma is called linear, where the rough patch is in a line or band across the skin.
• Systemic. This variation of the condition is exceptionally rare in children and quite serious. It can affect the entire body, including internal organs, and is referred to as Crest syndrome. This kind of scleroderma is diagnosed as either limited or diffuse depending on how quickly symptoms begin and progress./li>

The symptoms of scleroderma will vary depending on the type of scleroderma and the areas affected. Localized scleroderma (morphea) looks like patches of thickened skin that may be lighter or darker than the surrounding area. Your child may also complain that the skin feels tight and experience some joint problems.

Systemic scleroderma (Crest syndrome) can have a wide variety of symptoms, especially as the disease progresses. These can include the following:

• Thick, swollen fingertips
• Red spots on the hands and face
• Joint pain and mobility problems
• Spider veins
• Bumps or calcium spots on the bones of the fingers
• Stiff or sore fingers and knuckles
• Difficulty swallowing and heartburn
• Shortness of breath
• Irregular heart rhythms or heart failure
• Kidney problems
• Seizures

If your child is experiencing any of the symptoms of scleroderma, contact your child’s healthcare provider to schedule an appointment. You should seek immediate emergency care for your child if you observe more serious symptoms like shortness of breath, heart problems, and seizures.

It’s unclear what causes scleroderma, although doctors believe environmental factors have some role in the development of autoimmune disorders. In scleroderma, the body appears to produce too much collagen [kol-uh-juh n], the protein responsible for the elasticity of the skin. The body then begins to attack the healthy connective tissue, causing scarring. In localized scleroderma, this causes skin damage. In systemic scleroderma, it can mean internal damage to organs.

There also seems to be a genetic link to scleroderma, but it is not one inherited from direct family members and is still being researched. People of European descent are more likely to develop scleroderma.

Your child’s doctor will do a physical exam and discuss your child’s medical history if scleroderma is suspected. Most of the time, the first step is to test for antibodies to determine if your child’s immune system is working normally. Other tests to confirm the diagnosis may include the following.

• Blood and urine tests
• ECG (electrocardiogram [ih-LEK-trow-KAR-dee-oh-GRAM]
• Echocardiogram [eh-koh-KAR-dee-oh-GRAM])
• X-ray

Sometimes, scleroderma can be confused with a different condition called lupus. Both are autoimmune disorders that attack healthy tissue and share similar symptoms. One of the main differences between scleroderma and lupus is that people with scleroderma do not have increased sensitivity to light.

Because the cause of scleroderma is not well understood, there is no way to prevent this degenerative condition. You can help slow the progression of scleroderma in your child by following a treatment plan and making some changes to promote a healthier lifestyle. A few tips that will manage symptoms and offer a better quality of life include the following.

• Encourage your child to stay active.
• Follow your child’s treatment plan closely
• Make sure your child eats a healthy diet and avoid foods that might trigger inflammation

Talk with your child’s doctor about steps you can take to promote a healthy lifestyle. With the proper support and medical care, most children with scleroderma can expect a good prognosis and a normal lifespan.

Because the cause of scleroderma is not well understood, there is no way to prevent this degenerative condition. You can help slow the progression of scleroderma in your child by following a treatment plan and making some changes to promote a healthier lifestyle. A few tips that will manage symptoms and offer a better quality of life include the following.

• Encourage your child to stay active.
• Follow your child’s treatment plan closely
• Make sure your child eats a healthy diet and avoid foods that might trigger inflammation

Talk with your child’s doctor about steps you can take to promote a healthy lifestyle. With the proper support and medical care, most children with scleroderma can expect a good prognosis and a normal lifespan.

Scleroderma [skleer-uh-DUR-muh], which means “hard skin,” is a chronic disease that occurs rarely in children. The most common form results in hard patches of skin in one area of the body.