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. Tests Examination of the skin may show tightness, thickening, and hardening. The ESR is elevated. The rheumatoid factor is elevated. An antinuclear antibody test is positive.