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Systemic scleroderma is a rare disease, with an annual incidence that varies in different populations. Estimates of incidence (new cases per million people) range from 3.7 to 43 in the United Kingdom and Europe, 7.2 in Japan, 10.9 in Taiwan, 12.0 to 22.8 in Australia, 13.9 to 21.0 in the United States, and 21.2 in Buenos Aires. [ 48 ]
As of 2012, the five-year survival rate for systemic scleroderma was about 85%, whereas the 10-year survival rate was just under 70%. [44] This varies according to the subtype; while localized scleroderma rarely results in death, the systemic form can, and the diffuse systemic form carries a worse prognosis than the limited form.
Scleroderma (systemic sclerosis) Skin, organs ANA, anti-Scl-70, anti-centromere Confirmed 240 per 1,000,000 [22] Vitiligo: Skin Various, not specific Confirmed 1%
Linear scleroderma generally first appears in young children. [3] Frontal linear scleroderma (also known as en coup de sabre or morphea en coup de sabre) is a type of linear scleroderma characterized by a linear band of atrophy and a furrow in the skin that occurs in the frontal or frontoparietal scalp.
Micrograph of subepithelial sclerosus (middle third of image) in a case of lichen sclerosus. H&E stain . Sclerosis (from Ancient Greek σκληρός (sklērós) 'hard') is the stiffening of a tissue or anatomical feature, usually caused by a replacement of the normal organ -specific tissue with connective tissue .
Sclerodactyly is often preceded by months or even years by Raynaud's phenomenon when it is part of systemic scleroderma. [citation needed] The term "sclerodactyly" comes from Greek skleros 'hard' and daktylos 'digit'. It is generally associated with systemic scleroderma and mixed connective tissue disease, and auto-immune disorders.
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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.