WHAT IS SCLERODERMA?

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Scleroderma is a widespread connective tissue disease. It causes the skin and other body parts to slowly degenerate, thicken, and stiffen.

What is going on in the body?

Scleroderma results from an overproduction of collagen. This is the main supportive protein in the body. It affects tiny blood vessels and almost every organ. A person may develop either a local or a systemic form of scleroderma. The local form occurs in the skin and soft tissues below the skin. The systemic form affects body organs and structures, too, such as the: heart lungs kidneys gastrointestinal (GI) tract esophagus This condition usually occurs between ages 20 and 40. It is more common in women than men, but can affect either sex. About 300,000
people in the US have scleroderma.

What are the signs and symptoms of the condition?

Signs and symptoms include: numbness, pain, or color changes in the fingers, toes, cheeks, nose, and ears. These may occur spontaneously. Or they may be brought on by cold or emotional distress, a condition called http://health.discovery.com/diseasesandcond/encyclopedia/1737.html
Raynaud's phenomenon. sores or ulcers, especially on the fingers and toes,due to poor circulation slow hardening and tightening of the skin,especially on the arms, face, and hands. This hampers flexibility. Skin may appear shiny on the hands and forearms. calcium deposits in the skin and muscles dilated blood vessels especially on the hands, face, and chest stiffness or
http://health.discovery.com/diseasesandcond/encyclopedia/3147.html pain in the joints puffy hands and feet, particularly in the morning http://health.discovery.com/diseasesandcond/encyclopedia/3099.html
shortness of breath
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high blood pressure
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headaches swelling in the legs and ankles dry eyes and mouth digestive problems, such as trouble swallowing, poor food absorption, bloating after eating, weight loss, and heartburn What are the causes and risks of the condition?The cause of scleroderma is unknown, but it appears to be an
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autoimmune disorder. People heavily exposed to certain chemicals or substances may be at higher than normal risk for scleroderma. These substances include: silica plastics detergent herbicides silicone prostheses various drugs, such as cocaine and amphetamines Mild cases may develop from occupational repetitive stresses on the hand, such as from working with a jackhammer.

What can be done to prevent the condition?

There is no known way to prevent scleroderma.

How is the condition diagnosed?

The diagnosis is made after a medical history and physical exam.
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Raynaud's phenomenon may be a sign of scleroderma. A
http://health.discovery.com/diseasesandcond/encyclopedia/1073.html biopsy may help diagnose this condition. During a biopsy, a tissue sample of the affected skin is removed for laboratory study. Other diagnostic procedures may include: blood tests tests of heart, lung, and GI tract function

What are the long-term effects of the condition?

There is no cure for scleroderma at this time. In a minority of people, problems involving the
organs can cause:
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high blood pressure lung problems kidney failure intestinal problems that may lead to
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malnutrition Death may occur from gastrointestinal, heart, kidney, or lung involvement.

What are the risks to others?

There are no risks to others. This condition is not contagious.

What are the treatments for the condition?

Treatment depends on symptoms and severity. Medications used to treat this disorder include: corticosteroids to reduce inflammation relaxin to help slow production of collagen and loosen existing excess collagen vasodilators to relax and dilate blood vessels calcium channel
blockers to treat
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Raynaud's phenomenon slow-acting antirheumatic medications, such as hydroxychloroquine or penicillamine immunosuppressive medications, such as methotrexate, azathioprine, and cyclophosphamide. These medications suppress the immune response, thereby reducing inflammation. antacids to relieve heartburn antibiotics to fight infection nitroglycerine ointment
to treat hardened skin. This relaxes smooth muscle and dilates the arteries.
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Psychotherapy or counseling may help the person adjust to living with an
incurable disease. There are support groups for this disorder. Joining one
may help the person to deal with the illness.

What are the side effects of the treatments?

Corticosteroids may cause: weight gain
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high blood pressure acne easy
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bruising bone loss, leading to
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osteoporosis high blood sugar a higher risk of infection,
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cataracts,
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glaucoma, and muscle
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weakness Immunosuppressive medications may cause: a higher risk of infection stomach upset a decrease in liver and kidney function Antibiotics may cause: stomach upset diarrhea
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allergic reaction

What happens after treatment for the condition?

The courseof the disorder varies unpredictably. A person who only has the local form
of scleroderma that affects skin has a better prognosis.

How is the condition monitored?

A person with scleroderma should have frequent physical exams by his or her healthcare provider. This helps the provider monitor the activity of the disorder and determine possible complications. Frequent blood tests may help monitor the disorder as well.