Systemic sclerosis (scleroderma)

Alternative names:

CREST syndrome; progressive systemic sclerosis; scleroderma

Definition:

A diffuse connective tissue disease characterized by fibrotic, degenerative, and inflammatory changes in the skin, blood vessels, skeletal muscles, and internal organs.

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 is also thought to occur. In the skin, ulceration, calcification, and changes in pigmentation may occur. Systemic features may include fibrosis and atrophy 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. The incidence is 2 out of 10,000 people.