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Scleroderma

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Scleroderma facts

  • Scleroderma is a disease of the connective tissue featuring skin thickening that can involve scarring, blood vessel problems, varying degrees of inflammation, and is associated with an overactive immune system.
  • CREST syndrome is a limited form of systemic sclerosis.
  • Patients with scleroderma can have specific antibodies (ANA, anticentromere, or antitopoisomerase) in their blood that suggest autoimmunity.
  • Treatment of scleroderma is primarily directed toward the particular individual's symptoms.

What is scleroderma?

Scleroderma is an autoimmune disease of the connective tissue characterized by skin thickening, spontaneous scarring, blood vessel disease, and varying degrees of inflammation, associated with an overactive immune system. Autoimmune diseases are illnesses that occur when the body's tissues are attacked by its own immune system. Scleroderma is characterized by the formation of scar tissue (fibrosis) in the skin and, potentially, in the organs of the body. This leads to thickness and firmness of involved skin.

Scleroderma is classified as localized or widespread (systemic sclerosis with a tendency to affect internal organs). Systemic sclerosis is further divided into limited and diffuse based upon the extent of skin involvement.

What causes scleroderma?

The cause of scleroderma is not known. Researchers have found some evidence that certain genes are important hereditary factors, but the environment seems to also play a role. The result is activation of the immune system in a susceptible individual, causing damage to the inner lining of tiny blood vessels and injury to tissues that result in scar tissue formation and the accumulation of excess collagen.

The fact that genes seem to cause a predisposition to developing scleroderma means that inheritance at least plays a partial role. It is not unusual to find other autoimmune diseases in families of scleroderma patients. Some evidence for the role genes may play in leading to the development of scleroderma comes from the study of Choctaw Native Americans who are the group with the highest reported prevalence of the disease. The disease is more frequent in females than in males.

Medically Reviewed by a Doctor on 12/10/2014

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Source: MedicineNet.com
http://www.medicinenet.com/scleroderma/article.htm

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