Scleroderma

Scleroderma is a rare, chronic disease characterized by excessive deposits of collagen in the skin or other organs. Progressive systemic scleroderma or systemic sclerosis, the generalised type of the disease, can be fatal. The localised type of the disease tends not to be fatal.

Scleroderma affects the skin, and in more serious cases it can affect the blood vessels and internal organs. The most evident symptom is the hardening of the skin and associated scarring. Typically, the skin appears reddish or scaly in appearance. Blood vessels may also be more visible. Where large areas are affected, fat and muscle wastage will weaken limbs and affect appearance.

The seriousness of the disease varies hugely between cases. The two most important factors to consider are the level of internal involvement and the total area covered by the disease. For example, there have been cases where the patient has no more than one or two lesions, perhaps covering a few inches. Less serious cases tend not to involve the internal bodily functions.

There is no clear obvious cause for scleroderma and systemic sclerosis. Genetic predisposition appears to be limited: genetic concordance is small; still, there often is a familial predisposition for autoimmune disease. Polymorphisms in COL1A and TGF-ß1 may influence severity and development of the disease. There is limited evidence implicating cytomegalovirus as the original epitope of the immune reaction, and organic solvents and other chemical agents have been linked with scleroderma.

There is no cure for every patient with scleroderma, though there is treatment for some of the symptoms, including drugs that soften the skin and reduce inflammation. Some patients may benefit from exposure to heat.  A range of NSAIDs can be used to ease symptoms, such as naproxen. If there is esophageal dysmotility, care must be taken with NSAIDs as they are gastric irritants, and so a proton pump inhibitor such as omeprazole can be given in conjunction.  Immunosuppressant drugs, such as mycophenolate mofetil or cyclophosphamide are sometimes used to slow the progress.

...More at Wikipedia

Related Links:

• Scleroderma Foundation
• US National Library of Medicine
• Mayo Clinic

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