Systemic sclerosis (scleroderma)

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CREST syndrome

Raynaud's phenomenon

Sclerodactyly

Telangiectasia

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

CREST syndrome; Progressive systemic sclerosis; Scleroderma

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: 04/20/2005
Reviewed By: Stanford Peng, M.D., Ph.D., Division of Rheumatology, Washington University School of Medicine, St. Louis, MO. Review provided by VeriMed Healthcare Network.