Scleroderma

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 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

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.
Urinalysis shows protein and microscopic blood.
A chest X–ray may show fibrosis.
Pulmonary function studies often show restrictive lung disease.
A skin biopsy may also be performed.

Complications

Cardiac failure.
Renal failure.
Pulmonary fibrosis.
Malabsorption (inadequate absorption of nutrients from the intestinal tract).

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