Introduction
The name scleroderma is appropriately derived from the Greek word skleros, which means hard, and derma, which means skin. The disease is categorized as a rheumatologic disorder because it affects the connective tissues in the body. Scleroderma is a rare disease marked by the following:
- Damage to the cells lining the walls of small arteries.
- An abnormal build-up of tough scar-like fibrous tissue in the skin.
Patients with scleroderma may develop either a localized or a systemic (body-wide) form of the disease.
Localized Scleroderma
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Localized scleroderma usually affects only the skin on the hands and face. Its course is very slow and it rarely, if ever, becomes systemic or causes severe complications. There are two primary forms of localized scleroderma: morphea and linear scleroderma.
Morphea Scleroderma. In morphea scleroderma patches of hard skin form and can persist for years. Eventually, however, they may improve or even disappear. There is less than a 1% chance that this disorder will progress to systemic scleroderma.
Linear Scleroderma. Linear scleroderma causes bands of hard skin across the face or on a single arm or leg. Linear scleroderma may also involve muscle or bone. In rare cases, if this variant affects children or young adults, it may impair growth and cause severe deformities in the arms and legs.
Systemic Scleroderma
In systemic scleroderma (also called systemic sclerosis) the organs of the body, widespread areas of the skin, or both may be involved. This form of scleroderma has two primary variants: limited (also called CREST syndrome) or diffuse scleroderma. Both forms are progressive, although most often the course in either one is slow.
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