Definition of Scleroderma

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Scleroderma: A disease of connective tissue with the formation of scar tissue (fibrosis) in the skin and sometimes also in other organs of the body.

Scleroderma is classified into diffuse and limited forms. The CREST syndrome is a limited form of scleroderma. CREST stands for:

• C ... Calcinosis (the formation of tiny deposits of calcium in the skin),
• R ... Raynaud phenomenon (spasm of the tiny artery vessels supplying blood to the fingers, toes, nose, tongue, or ears),
• E ... Esophagus (esophageal involvement by the scleroderma),
• S ... Sclerodactyly (localized thickening and tightness of the skin of the fingers or toes) and
• T ... Telangiectasias (dilated capillaries that form tiny red areas, frequently on the face, hands and in the mouth behind the lips).

The cause of scleroderma is not known. There is some evidence that genes are important factors. This means that inheritance at least play a partial role in causing the disease. It is not unusual to find other autoimmune diseases in families of scleroderma patients. The disease is more frequent in females than in males.

People with scleroderma can have antibodies in their blood reflecting autoimmunity (in which the immune system attacks the skin and other parts of the body).

The treatment of scleroderma is directed toward the individual feature that are most troubling.