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Scleroderma
The disease is also categorized as a rheumatologic disorder because it affects the connective tissues in the body. It's a disease of the connective tissue, i.e. characterized by the formation of scar tissue in the skin and organs of the body leading to thickness and firmness of involved areas. Scleroderma is a rare disease that damages to the cells lining the walls of small arteries and an abnormal build-up of tough scar-like fibrous tissue in the skin. The name scleroderma is derived from the Greek word skleros, which means hard, and derma, which means skin. The cause of scleroderma is not known. Researchers have shown evidences that the genes are important factors, but the environment seems to also play a role. The result is activation of the immune system causing injury to tissues that result in injury similar to scar tissue formation. Inheritance plays a partial role to developing scleroderma. The disease is more frequent in females than in males.
Causes and Risk Factors
The cause of scleroderma is unknown. Scleroderma appears to occur as an autoimmune response, in which a defective immune system attacks or sets conditions that damage the body itself. In scleroderma, this response produces inflammation and an overproduction of collagen, the tough fibrous protein that helps construct connective tissues. Most likely this disease process is regulated by a number of genetic defects as well as environment which plays a crucial role. Scleroderma is neither contagious nor is it passed on from one generation to the next, except in rare circumstances.
Classification
Scleroderma has been categorized on basis of the degree and location of the skin involvement. The two major classifications are the generalized scleroderma and localized scleroderma. Generalized scleroderma refers to the scleroderma that affects many parts of the body while localized scleroderma affects mainly the skin. The generalized scleroderma and localized are further subdivided. The diffuse and limited are subdivisions of the generalized one whereas morphia and linear are the subdivisions of localized scleroderma.
- The diffuse form of scleroderma is involves symmetric thickening of skin of the extremities, face, trunk that can rapidly progress to hardening from an initial inflammatory phase.
- In the limited form of scleroderma tends to be confined to the skin of the fingers and face. The skin changes and other features of disease tend occur slowly than in diffuse form.
- The morphia refers to oval shaped, hard whitish patches on the skin.
- The linear refers to the lines or streaks of thickened skin in areas such as arms, legs or forehead.
Diagnosis
The doctor will first examine the skin, especially on your fingers, hands and face. If he feels it to be a case of scleroderma he or she may ask you to go for blood tests as well as skin biopsy. In biopsy, a small sample of skin will be removed and examined in a laboratory. If scleroderma affects internal organs such as the heart, lungs or digestive organs, a chest X-ray and other tests may be recommended.
Prevention
There is no way for the prevention of Scleroderma.
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