Scleroderma, Systemic
| Definition: |
A chronic multi-system disorder of CONNECTIVE TISSUE. It is characterized by SCLEROSIS in the SKIN, the LUNGS, the HEART, the GASTROINTESTINAL TRACT, the KIDNEYS, and the MUSCULOSKELETAL SYSTEM. Other important features include diseased small BLOOD VESSELS and AUTOANTIBODIES. The disorder is named for its most prominent feature (hard skin), and classified into subsets by the extent of skin thickening: LIMITED SCLERODERMA and DIFFUSE SCLERODERMA. |
| Notes: |
note specific indentations and SCLERODERMA, LOCALIZED |
Scleroderma, Systemic Categories.
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Scleroderma, Diffuse - A rapid onset form of SYSTEMIC SCLERODERMA with progressive widespread SKIN thickening over the arms, the legs and the trunk, resulting in stiffness and disability. |
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Scleroderma, Limited - The least progressive form of SYSTEMIC SCLERODERMA with skin thickening restricted to the FACE; NECK; and areas distal to the elbows and/or knees, sparing the trunk. The CREST SYNDROME is a form of limited scleroderma. |
Scleroderma, Systemic Definitions and Terms
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