Scleroderma: A disease of connective tissue that causes scar tissue (fibrosis) to form in the skin and sometimes also in other organs of the body. Scleroderma is classified into diffuse and limited forms. Diffuse scleroderma affects many internal and external areas of the body, including the skin of the entire body, the bowels, and the lungs. Limited scleroderma affects only certain body sections. The CREST syndrome is the most common limited form of scleroderma. CREST stands for
- CCalcinosis (the formation of tiny deposits of calcium in the skin)
- RRaynaud phenomenon (spasms of the tiny arteries that supply blood to the fingers, toes, nose, tongue, or ears)
- EEsophagus (esophageal involvement by the scleroderma)
- SSclerodactyly (localized thickening and tightness of the skin of the fingers or toes)
- TTelangiectasias (dilated capillaries that form tiny red areas, frequently on the face, on the hands, and in the mouth, behind the lips)
The cause of scleroderma is unknown. There is some evidence that genes play at least a partial role in causing the disease. The immune system plays a central role in the disease process. It is not unusual to find other autoimmune diseases in families of scleroderma patients. The treatment of scleroderma is directed toward the particular organ system that is causing symptoms. The disease occurs more frequently in females than in males.