Scleroderma is one of several diseases that affect the connective tissues in the body. The name scleroderma comes from the Greek words "skleros," meaning "hard," and "derma," meaning "skin." Thus, scleroderma literally means "hard skin." This term refers to the primary symptom of the disease.

As scleroderma progresses, the skin becomes thick. Other organs may also be affected. The severity of the disease depends on the type of scleroderma present. There are two basic categories, localized and systemic.

Localized scleroderma affects only the skin. There are two main forms of local scleroderma: morphea and linear.

Morphea scleroderma

With morphea scleroderma, hard, oval-shaped patches form on the skin. The patches are usually whitish with a purple ring around them and are most commonly seen on the trunk, but can occur anywhere. Although the patches can persist for years, they may also improve or disappear spontaneously.

Linear Scleroderma

With linear scleroderma, bands of hard skin form across the face or on a single arm or leg. Linear scleroderma can sometimes damage the growth of a muscle or bone beneath the hardened skin area and can affect mobility in the limb or extremity.

Systemic scleroderma is sometimes called generalized scleroderma or Progressive Systemic Sclerosis (PSS). This form of the disease can affect all areas of the skin, blood vessels, and the internal organs. Systemic scleroderma has two primary forms, limited and diffuse.

Limited Scleroderma, or CREST Syndrome

About 50 percent of patients with systemic scleroderma have the limited form. Limited scleroderma usually has a very gradual onset. In some cases, the first symptom, which is typically a blood vessel condition called Raynaud's phenomenon, may appear five to ten years before the full syndrome develops. Initially, the disease may only affect the skin on the face and hands. As the disease progresses, it can reach organs such as the esophagus and lungs. Limited scleroderma is sometimes called CREST syndrome. CREST is an acronym for the diseases's pattern of symptoms, which include:

Calcinosis, or the formation of mineral deposits under the skin. The deposits usually occur around pressure areas (elbows) or joints. Skin ulcers may form over the deposits. The ulcers may drain calcium deposits and can become infected.

Raynaud's phenomenon, a condition in which the blood vessels constrict, interfering with blood flow. The arteries of the fingers are commonly involved.

Esophagus dysfunction, or scarring of the muscles in the esophagus. The scar tissue prevents the muscles from working properly, and problems with swallowing may result. Stomach acid can also flow back into the esophagus and cause esophageal ulceration and further scarring.

Sclerodactylia, also called acrosclerosis, a condition in which the skin of the fingers becomes thickened and stiff.

Telangiectasia, a condition in which the small blood vessels dilate or enlarge, producing flat red marks on the hands, face, and tongue.

Diffuse Scleroderma

Diffuse scleroderma usually develops more quickly than limited scleroderma and can affect all areas of the skin. Patients with this form of scleroderma also experience esophageal problems and Raynaud's phenomenon.

Gastrointestinal, lung, heart, and kidney problems often occur as the disease progresses.

References

First published April 1, 2000
Last updated September 10, 2003
Copyright © 2000 Accordant Health Services, Inc. All Rights Reserved.

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