|
Scleroderma is a chronic autoimmune disease which characterized by excessive deposits of collagen in the skin or other organs and thickening of the skin. Localized scleroderma affects the skin in limited areas and the musculoskeletal system. Scleroderma is a chronic disease and in generalized form (systemic sclerosis) can be fatal as a result of heart, kidney, lung or intestinal damage.
Most people with scleroderma have cold-induced spasms of small blood vessels in their hands or feet, known as Reynaud’s phenomenon. Formation of calcium deposits is also common in systemic scleroderma, and is often seen near the elbows, knees or other joints. Patients with larger amounts of cutaneous involvement are more likely to have involvement of the internal tissues and organs.
In most cases, the cause of scleroderma is unknown. Systemic sclerosis is associated with over-activation of the immune system. This causes damage to cells that line small blood vessels, which in turn leads to the over-production of scar tissue.
Diagnosis of scleroderma is based on clinical history and physical findings. Laboratory, X-ray, biopsy and pulmonary function tests determine the extent and severity of internal organ involvement.
There is no known cure for scleroderma. A range of anti-inflammatory drugs can be used to ease symptoms. Organ-specific treatments for scleroderma are also used. Hematopoietic stem cell transplantation is being studied in patients with severe systemic sclerosis. In those studies improvement in life expectancy and severity of skin changes has been noted. |