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Systemic sclerosis (scleroderma)

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Contents of this page:

Illustrations

Raynaud's phenomenon
Raynaud's phenomenon
CREST syndrome
CREST syndrome
Sclerodactyly
Sclerodactyly
Telangiectasia
Telangiectasia

Alternative names    Return to top

CREST syndrome; Progressive systemic sclerosis; Scleroderma

Definition    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

Additional symptoms that may be associated with this disease:

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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