What Is Scleroderma?
Scleroderma (pronounced SKLEER-oh-der-ma) is Greek for hard skin. It's classified as both a connective-tissue disorder and a rheumatic condition. In scleroderma, the skin gradually tightens and thickens or hardens. It loses its ability to stretch. Tiny blood vessels throughout the body also may be affected, causing widespread damage to the internal organs.
Although the disease usually affects the hands, face, and feet, impaired blood flow can eventually injure the body's digestive, respiratory, and circulatory systems.
Scleroderma can look very different in different people. There are many subtypes of this condition. Doctors generally classify scleroderma as either localized or systemic, depending on the degree to which the skin is affected. The widespread form of the disease is often called systemic sclerosis. It can be life threatening.
An estimated 300,000-500,000 Americans have scleroderma. The incidence varies across geographic regions, and appears to be higher in the U.S. than in Europe or Japan. No one race or ethnic group is affected more than another.
Women are affected in much greater numbers; they develop localized forms of the disease three times as frequently as men. More than 80% of people with systemic scleroderma are women aged 30 to 50. Rarely, children can also develop scleroderma. In the past two decades, longevity has improved for people with systemic scleroderma. Eighty percent survive five years or more.
Some evidence for the role genes may play in leading to the development of scleroderma comes from the study of Choctaw Native Americans who are the group with the highest reported prevalence of the disease. The disease is more frequent in females than in males.
What Causes It?
The underlying cause of scleroderma remains unclear. The main feature of the disease is excessive production of collagen. Collagen is one of the building blocks from which skin is made. Usually it makes the skin supple. But when too much collagen accumulates it causes various tissues to become thick and to lose flexibility. Current research focuses on the interplay between the immune system, the vascular (blood vessel) system, and the growth of connective tissue.
What Are the Symptoms?
The symptoms and manifestations of scleroderma vary widely between individuals. The most common symptom is tightening and hardening or thickening of the skin of the arms, legs, hands, feet, and face. The skin continues to thicken during the first two to three years of the disease, after which thickening ceases and may even recede.
Symptoms of scleroderma may include:
- Gradual tightening and thickening of the skin
- Swelling, stiffness, or pain in the fingers, toes, hands, feet, or face
- Tingling, numbness, or puffiness
- Skin discoloration
- Small white bumps under the surface of the skin
- Cold sensitivity and a bluish or reddish tint in the hands and feet (called Raynaud's Syndrome)
- Telangiectasias (red spots from permanently dilated tiny blood vessels) on the fingers, palms, face, lips, or tongue
- Ulcers or sores on fingertips, knuckles, or elbows
- Brittle bones that may easily break
- Loss of the skin's ability to stretch
- Muscle weakness
- Curling of the fingers
- Digestive problems (heartburn and trouble swallowing)
- Loss of hand function because of skin tightening on fingers and hands
- Shortness of breath, possibly from heart or lung damage
Five particular symptoms that sometimes occur together have been clinically recognized as a variation of scleroderma called CREST syndrome. The acronym CREST stands for calcinosis (painful calcium deposits in the skin), Raynaud's phenomenon (abnormal blood flow in the hands and feet in response to cold or stress), esophageal dysfunction (problems with swallowing caused by internal scarring), sclerodactyly (tightening of the skin on the fingers or toes), and telangiectasia on the hands, palms, forearms, face, and lips. People with CREST syndrome generally have a relatively mild form of systemic scleroderma.
Call Your Doctor If:
- You notice tightening and thickening of your skin.
- You have unexplained swelling of the fingers, toes, hands, feet, or face.
- You have tingling, numbness, puffiness, or skin discoloration.
- You have small white bumps under the surface of your skin.
- You develop sensitivity to cold in your hands and feet.
- You develop red spots on your fingers, palms, face, lips, or tongue.
- You get ulcers or sores on your fingertips, knuckles, or elbows.
What Are the Treatments?
There is no cure for scleroderma. Treatment focuses on relieving symptoms and reducing the risk of complications.
Vasodilators, medicines that relax and open the blood vessels, may be prescribed to relieve Raynaud's phenomenon. Avoiding exposure to cold and sunlight is also helpful. Drugs called angiotensin converting enzyme (ACE) inhibitors and other blood pressure medications can help control blood pressure and may be used to alleviate serious kidney complications. Non-steroidal anti-inflammatory drugs (NSAIDs), such as aspirin, naproxen and ibuprofen, may ease joint pain, and antibiotics may be prescribed for intestinal problems.
D-penicillamine is thought to decrease the production of collagen and may delay the progress of the disease. Steroids, such as cortisone or prednisone, may be used to decrease inflammation.
Stretching and physical therapy may help keep joints and tissue strong and flexible. Raynaud's phenomenon can be relieved somewhat by staying warm. Wearing gloves and warm socks will help.
Rarely, orthopedic hand or cosmetic surgery may be necessary to correct severe joint contractures, skin deformities, or scars. Usually before surgery can be performed, the disease must be in remission for at least five years
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