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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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