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Systemic scleroderma, or systemic sclerosis, is an autoimmune rheumatic disease characterised by excessive production and accumulation of collagen, called fibrosis, in the skin and internal organs and by injuries to small arteries. There are two major subgroups of systemic sclerosis based on the extent of skin involvement: limited and diffuse ...
Scleroderma is caused by genetic and environmental factors. [ 4 ] [ 5 ] [ 17 ] [ 18 ] Mutations in HLA genes seem to play a crucial role in the pathogenesis of some cases; likewise silica , aromatic and chlorinated solvents, ketones , trichloroethylene , welding fumes, and white spirits exposure seems to contribute to the condition in a small ...
Scleromyositis, is an autoimmune disease (a disease in which the immune system attacks the body). People with scleromyositis have symptoms of both systemic scleroderma and either polymyositis or dermatomyositis, and is therefore considered an overlap syndrome.
Each disorder is listed with the primary organ or body part that it affects and the associated autoantibodies that are typically found in people diagnosed with the condition. Each disorder is also categorized by its acceptance as an autoimmune condition into four levels: confirmed, probable, possible, and uncertain.
Anti Scl-70 antibodies (also called anti-topoisomerase I after the type I topoisomerase target [1]) is a type of antinuclear autoantibody seen mainly in diffuse systemic scleroderma, but is also seen the more limited form of systemic scleroderma called CREST syndrome. [2]
[5] [3] Genetic testing may rarely be done to support the diagnosis. [2] There is no known prevention or cure. [1] [2] Surgery may be done to remove tumors that are causing problems or have become cancerous. [1] Radiation and chemotherapy may also be used if cancer occurs. [1]
This disease may cause white or yellow-ish spots on the arms or legs. The syndrome, a special case of scleroderma, is named after the American physician, Telfer B. Reynolds, MD (1921–2004), who first described it. He is also known for creating one of the world's first hepatology programs at the University of Southern California.
Morphea is a form of scleroderma that mainly involves isolated patches of hardened skin on the face, hands, and feet, or anywhere else on the body, usually with no internal organ involvement. [1] However, in Deep Morphea inflammation and sclerosis can be found in the deep dermis, panniculus, fascia, superficial muscle and bone. [2]: 130