|Terms & Properties|
Concept Unique Identifier (CUI): C0011644
NCI Thesaurus Code: C26746 (see NCI Thesaurus info)
Semantic Type: Disease or Syndrome
NCIt Definition: A localized or systemic chronic and progressive autoimmune disorder characterized by thickening of the skin and the connective tissues. Localized scleroderma affects only the skin. Systemic scleroderma affects internal organs, including the heart, lungs, gastrointestinal tract, and kidneys.
GARD Definition: Scleroderma is a group of widespread connective tissue diseases that involve changes in the skin, blood vessels, muscles, and internal organs. There are two main types. Localized scleroderma affects only the skin. Systemic scleroderma affects the blood vessels and internal organs, as well as the skin. Although the cause of scleroderma is unknown, it is believed to be related to an overproduction and accumulation of collagen which results when the immune system turns against the body (autoimmune reaction). There is no cure, but various treatments can relieve symptoms. - this information is from GARD/ORDR/NCATS.
HPO Definition: A chronic autoimmune phenomenon characterized by fibrosis (or hardening) and vascular alterations of the skin. [HPO:sdoelken]
Scleroderma means hard skin. It is a group of diseases that cause abnormal growth of connective tissue. Connective tissue is the material inside your body that gives your tissues their shape and helps keep them strong. In scleroderma, the tissue gets hard or thick. It can cause swelling or pain in your muscles and joints.
Symptoms of scleroderma include
No one knows what causes scleroderma. It is more common in women. It can be mild or severe. Doctors diagnose scleroderma using your medical history, a physical exam, lab tests, and a skin biopsy. There is no cure, but various treatments can control symptoms and complications.
NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases
NCI-GLOSS Definition: A chronic disorder marked by hardening and thickening of the skin. Scleroderma can be localized or it can affect the entire body (systemic).
NICHD Definition: A chronic and progressive autoimmune disorder characterized by thickening of the skin and the connective tissues.
CSP Definition: chronic hardening and thickening of the skin caused by swelling and thickening of fibrous tissue leading to eventual atrophy of the epidermis; can occur as a localized or a systemic disease.
Synonyms & Abbreviations: (see Synonym Details)
|Progressive systemic scleroderma|
External Source Codes:
|NCI Thesaurus Code||C26746 (see NCI Thesaurus info)|
|FDA_Table||Patient Code (Appendix B)||NCI|
|HPO_COMMENT||A chronic autoimmune disease characterized by fibrosis (or hardening), vascular alterations, and autoantibodies. Sclerosis of the skin occurs in association with disorders as diverse as dermatomyositis, Werner's syndrome, porphyria, muscle glycogenesis, primary systemic amyloidosis, melorheostosis, and malignancy including the carcinoid syndrome. This term is intended to represent the phenotypic feature scleroderma rather than a particular disease.||HPO|
|MP_PRIMARY_INSTITUTE_URL||National Institute of Arthritis and Musculoskeletal and Skin Diseases http://www.niams.nih.gov/||MEDLINEPLUS|
|PRIMARY_PATH||10039710$10074472$10010761$10028395$Scleroderma$Connective tissue disorders NEC$Connective tissue disorders (excl congenital)$Musculoskeletal and connective tissue disorders||MDR|
Additional Concept Data: (none)