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Scleroderma

Systemic sclerosis

Scleroderma is a rare, chronic disease characterized by excessive deposits of collagen. Progressive systemic scleroderma or systemic sclerosis, the serious type of the disease, can be fatal. The local type of the disease is not serious.

Pathophysiology

The cause of the disease is unknown. The overproduction of collagen is thought to result from an autoimmune dysfunction.

Epidemiology

Scleroderma affects approximately 300,000 people in the United States. It is four times as common in women than in men.

Signs and symptoms

Scleroderma affects the skin, and in serious, life-threatening cases it affects the blood vessels and internal organs. The most evident symptom is the hardening of the skin. There is discoloration of the hands and feet in response to cold. The seriousness of the disease depends on which organs, if any, are affected. If the heart, lungs, or kidneys are affected, the disease is generally fatal. Most patients (>80%) have Raynaud's phenomenon, a vascular sign in the fingers.

Therapy

There is no cure for scleroderma, though there is treatment for some of the symptoms, including drugs that soften the skin and reduce inflammation. Patients often benefit from exposure to heat.

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