Pediatric Scleroderma in Children

What causes pediatric scleroderma?

The exact cause of pediatric scleroderma is unknown. It seems to involve a disorder of the cells lining small blood vessels.

Scleroderma may also be related to defects in the immune system, in which the body’s normally protective defense system causes damage to its own tissues.

Normally, the immune system helps defend the body against infection. In patients with scleroderma, the immune system triggers other cells to produce too much collagen (a protein). This extra collagen is deposited in the skin and organs, which causes hardening and thickening (similar to the scarring process).

Scleroderma is not contagious, so people cannot “catch it” or pass it on to someone else.

Who is affected by pediatric scleroderma?

There are only 5,000 to 7,000 children with scleroderma in the United States. Only 1.5 percent of all people with scleroderma develop it before age 10, and 7 percent develop it between ages 10 and 19.

Two thirds of children who acquire scleroderma are female.

How is scleroderma diagnosed?

The diagnosis of scleroderma is often made by a rheumatologist (arthritis specialist) or dermatologist (skin specialist) based on a combination of factors:

  • Presence of symptoms and physical signs
  • Characteristic skin changes (including location, size, shape, color)
  • Results of a complete medical history and physical exam

Other tests may be used to confirm the diagnosis or to determine the severity of the disease, such as:

  • Skin biopsy of affected skin, in which a small tissue sample is removed for evaluation under a microscope
  • Blood tests in which autoimmune proteins and kidney function are measured in a laboratory

Additional tests for systemic sclerosis

  • X-rays which can show changes in the skin, bones and internal organs such as the lungs and intestines
  • Tests to evaluate the swallowing function of the esophagus (tube leading from the mouth to the stomach)
  • Pulmonary function test (breathing test) to make sure the lungs are working properly
  • Echocardiography (ultrasound of the heart) to look at the heart function

No single test or X-ray will prove the diagnosis of scleroderma, and not all children will need all these tests.

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