| 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 of 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.
Prevention
There is no known prevention. Minimize exposure to silica
dust and polyvinyl chloride.
Symptoms
Blanching, blueness, or redness of fingers and toes
in response to heat and cold (Raynaud's phenomenon), pain, stiffness,
and swelling of fingers and joints, skin thickening and shiny hands and
forearm skin is hard tight and mask-like facial skin ulcerations on fingertips
or toes, esophageal reflux or heartburn, difficulty swallowing, bloating
after meals, weight loss, diarrhea, constipation, shortness of breath,
wrist pain, wheezing, skin, abnormally dark or light joint pain, hair
loss, eye burning, itching & discharge.
Signs and Tests
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Examination of the
skin may show tightness, thickening and hardening.
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The ESR is elevated.
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The rheumatoid factor
is elevated.
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An antinuclear antibody
test is positive.
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Urinalysis shows
protein and microscopic blood.
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A chest X-ray may
show fibrosis.
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Pulmonary function
studies often show restrictive lung disease.
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A skin biopsy may
also be performed.
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Complications
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Cardiac failure
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Renal failure
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Pulmonary fibrosis
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Malabsorption (inadequate
absorption of nutrients from the intestinal tract).
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