What is scleroderma?

Scleroderma is a condition in which the skin becomes unusually thick and hard. It is an autoimmune disease that causes inflammation (swelling) in the skin. The swelling can trigger an overproduction of collagen cells, a fibrous protein that is a major part of many tissues in the body. This overproduction can lead to fibrosis, or scarring.

What are the types of scleroderma?

There are two broad types of scleroderma:

  • Localized scleroderma: This form of scleroderma affects skin in only some area of the body and only occasionally spreads to underlying muscles or bone. It rarely involves internal organs and does not change into the generalized forms of scleroderma.
  • Systemic scleroderma (systemic sclerosis): This form of scleroderma can cause generalized thickened skin in all parts of the body. In addition to skin changes, scar tissue could develop in internal organs such as the kidneys, heart, lungs and gastrointestinal tract. Children with systemic sclerosis have more widespread skin changes that can result in limited joint movement.

Raynaud’s phenomenon (white, blue and red color changes of the fingers and/or toes upon exposure to cold or stress) is present early in a child with systemic sclerosis, as well as fatigue, joint pain, difficulty swallowing, abdominal pain, heartburn, diarrhea and shortness of breath.

Children with systemic sclerosis should be checked often for high blood pressure, as well as lung, kidney, gastrointestinal and heart problems.

Who is affected by scleroderma?

There are only 5,000 to 7,000 children with scleroderma in the United States. It is more common in adults then children. Only two percent of all people with scleroderma develop the disease before age 10, and seven percent develop it between ages 10 and 19.

What causes pediatric systemic scleroderma?

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

It 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).

What are the signs and symptoms of pediatric systemic scleroderma?

The skin changes of systemic scleroderma can include:

  • Loss of the skin’s ability to stretch
  • Decreased hand function (because of skin tightening on fingers and hand)
  • Raynaud's phenomenon (abnormal sensitivity to cold), which is usually seen in the hands. Signs include color changes in the hands (white, blue, red), tingling, discomfort and decreased sensation.
  • Enlarged red blood vessels on the hands, face and around the nail beds (telangiectasias)
  • Calcium deposits in the skin or other areas (calcinosis)

Initially, the skin of the hands and feet appears swollen. Over time, the skin tightens and hardens and may appear to have ridges, depressed areas or small pits that are seen mostly at the fingertips.

Since there is not much pain, it is common for scleroderma to be present for quite some time before a parent or child becomes concerned.

Other signs and symptoms of systemic scleroderma from internal organ involvement may include:

  • Joint inflammation with stiffness and pain
  • Sores (ulcers), mostly on the fingertips
  • Digestive problems (heartburn, trouble swallowing, diarrhea, stomach cramps)
  • Respiratory problems (chronic cough, difficulty breathing, tiredness)
  • Kidney involvement can lead to hypertension
  • Fatigue (easily becoming tired)
  • Muscle weakness

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