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Alternative names Return to top
CREST syndrome; Progressive systemic sclerosis; SclerodermaDefinition Return to top
Scleroderma is a diffuse connective tissue disease characterized by changes in the skin, blood vessels, skeletal muscles, and internal organs.Causes, incidence, and risk factors Return to top
The cause of scleroderma is unknown. The disease may produce local or systemic symptoms. The course and severity of the disease varies widely in those affected.
Excess collagen deposits in the skin and other organs produce the symptoms. Damage to small blood vessels within the skin and affected organs also occurs. In the skin, ulceration, calcification, and changes in pigmentation may occur.
Systemic features may include fibrosis and degeneration of the heart, lungs, kidneys and gastrointestinal tract.
The disease usually affects people 30 to 50 years old. Women are affected more often than men. Risk factors are occupational exposure to silica dust and polyvinyl chloride.
Symptoms Return to top
Signs and tests Return to top
Examination of the skin may show tightness, thickening, and hardening.Treatment Return to top
See scleroderma treatment.Support Groups Return to top
See scleroderma - support group.Expectations (prognosis) Return to top
In the majority of those affected, the disease is progressive. In some, remission occurs with a slow progression. People who only have skin involvement have a better prognosis (probable outcome). Death may occur from gastrointestinal, cardiac, kidney, or pulmonary (lung) involvement.Complications Return to top
Calling your health care provider Return to top
Call for an appointment with your health care provider if symptoms of this disorder are present, or if you have scleroderma and symptoms become worse, or if new symptoms develop.Prevention Return to top
There is no known prevention. Minimize exposure to silica dust and polyvinyl chloride. Update Date: 8/6/2003 Updated by: Megan E. B. Clowse, M.D., M.P.H., Division of Rheumatology, Johns Hopkins Hospital, Baltimore, MD. Review provided by VeriMed Healthcare Network.
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Page last updated: 28 October 2004 |