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Raynaud's phenomenon
Raynaud's phenomenon
CREST syndrome
CREST syndrome
Sclerodactyly
Sclerodactyly
Telangiectasia
Telangiectasia
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Systemic sclerosis (scleroderma)

Definition:
Scleroderma is a diffuse connective tissue disease characterized by changes in the skin, blood vessels, skeletal muscles, and internal organs.

Alternative Names:
CREST syndrome; Progressive systemic sclerosis; Scleroderma

Causes, incidence, and risk factors:

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.



Review Date: 8/6/2003
Reviewed 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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