Conditions InDepth: Scleroderma
Scleroderma is a disease of the body’s connective tissue. Connective tissue is found throughout the body, providing support and form for organs and structures. Scleroderma is thought to be an autoimmune disorder—a condition in which the immune system mistakes the body’s own tissue for a foreign invader, attacking and damaging it. Researchers believe that the immune system’s interaction with the connective tissue causes an overproduction of collagen—a tough, hard protein that makes up tendons, bones, ligaments, and scar tissue. When this collagen is deposited in various places throughout the body, it causes hardening and stiffening.
The most common areas of the body affected by scleroderma are the skin, blood vessels, joints, and internal organs (heart, lungs, kidneys, and digestive system).
Scleroderma is classified as:
Localized Scleroderma—This primarily affects the skin. Localized scleroderma is further divided into:
- Morphea—Skin lesions are firm, at times oval, whitish or brownish plaques, surrounded by a purplish ring.
- Linear—Skin lesions appear as hardened streaks or lines along the arms, legs, or forehead.
Generalized Scleroderma —This is divided into:
- Limited—A gradually progressing form of scleroderma that initially causes skin thickening, but progresses to affect the internal organs.
- Diffuse—A more quickly progressing form of scleroderma that causes the skin to thicken throughout the body, and may also affect the internal organs.
Systemic Sclerosis Sine Scleroderma —This is a very rare form of scleroderma in which there are no skin manifestations, but the internal organs are affected.
Scleroderma is relatively rare, affecting about 150,000 Americans. Worldwide, there are about 500 cases of scleroderma in every one million people.
Harrison’s Principles of Internal Medicine. 15th ed. McGraw-Hill; 2001.
National Institute of Arthritis and Musculoskeletal and Skin Diseases website. Available at: http://www.niams.nih.gov/ .
Scleroderma Foundation website. Available at: http://www.scleroderma.org/ .
Last reviewed August 2008 by
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