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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, such as the heart, lungs, kidneys, and digestive system.
Scleroderma
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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. It 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.

Overlap Syndrome

Overlap syndrome occurs when a person has symptoms of two or more autoimmune diseases. The most common diseases include:
What are the risk factors for scleroderma?What are the symptoms of scleroderma?How is scleroderma diagnosed?What are the treatments for scleroderma?Are there screening tests for scleroderma?How can I reduce my risk of scleroderma?What questions should I ask my doctor?What is it like to live with scleroderma?Where can I get more information about scleroderma?

References

Iaccarino L, Gatto M, et al. Overlap connective tissue disease syndromes. Autoimmun Rev. 2013;12(3):363-373.

Systemic sclerosis. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed.ebscohost.com/about/about-us. Updated August 5, 2013. Accessed August 8, 2013.

Scleroderma. National Institute of Arthritis and Musculoskeletal and Skin Diseases website. Available at: http://www.niams.nih.gov/Health%5FInfo/Scleroderma/default.asp. Updated August 2012. Accessed August 8, 2013.

What is scleroderma? Scleroderma Foundation website. Available at: http://www.scleroderma.org/site/PageServer?pagename=patients%5Fwhatis. Accessed August 8, 2013.

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