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Scleroderma (CUI C0011644) Suggest changes to this concept
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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]

MEDLINEPLUS Definition: 

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

  • Calcium deposits in connective tissues
  • Raynaud's phenomenon, a narrowing of blood vessels in the hands or feet
  • Swelling of the esophagus, the tube between your throat and stomach
  • Thick, tight skin on your fingers
  • Red spots on your hands and face

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)
dermatosclerosis
Progressive systemic scleroderma
scleroderma

External Source Codes: 
NCI Thesaurus Code C26746 (see NCI Thesaurus info)

Other Properties: Property Definitions
Name Value Source
CASE_SIGNIFICANCE_ID 900000000000020002 SNOMEDCT_US
Contributing_Source FDA NCI
Contributing_Source MedDRA NCI
Contributing_Source NICHD NCI
DATE_CREATED 10/01/1999 MEDLINEPLUS
DATE_CREATED 2010-08-10T04:14:42Z HPO
DID 2716-6998 CSP
EXTERNALLY_DEFINED N LNC
FDA_Table Patient Code (Appendix B) NCI
HAS_GARD_PAGE true GARD
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
IS_RARE false GARD
IS_SPANISH false GARD
MP_HEALTH_TOPIC_URL https://www.nlm.nih.gov/medlineplus/scleroderma.html MEDLINEPLUS
MP_OTHER_LANGUAGE_URL Spanish https://www.nlm.nih.gov/medlineplus/spanish/scleroderma.html MEDLINEPLUS
MP_PRIMARY_INSTITUTE_URL National Institute of Arthritis and Musculoskeletal and Skin Diseases http://www.niams.nih.gov/ MEDLINEPLUS
NICHD_Hierarchy_Term Scleroderma NCI
PRIMARY_PATH 10039710$10074472$10010761$10028395$Scleroderma$Connective tissue disorders NEC$Connective tissue disorders (excl congenital)$Musculoskeletal and connective tissue disorders MDR
PRIMARY_SOC 10028395 MDR
PT_IN_VERSION 10.0 MDR
PT_IN_VERSION 10.1 MDR
PT_IN_VERSION 11.0 MDR
PT_IN_VERSION 11.1 MDR
PT_IN_VERSION 12.0 MDR
PT_IN_VERSION 12.1 MDR
PT_IN_VERSION 13.0 MDR
PT_IN_VERSION 13.1 MDR
PT_IN_VERSION 14.0 MDR
PT_IN_VERSION 14.1 MDR
PT_IN_VERSION 15.0 MDR
PT_IN_VERSION 15.1 MDR
PT_IN_VERSION 16.0 MDR
PT_IN_VERSION 16.1 MDR
PT_IN_VERSION 17.0 MDR
PT_IN_VERSION 17.1 MDR
PT_IN_VERSION 18.0 MDR
PT_IN_VERSION 18.1 MDR
PT_IN_VERSION 19.0 MDR
PT_IN_VERSION 19.1 MDR
PT_IN_VERSION 8.0 MDR
PT_IN_VERSION 8.1 MDR
PT_IN_VERSION 9.0 MDR
PT_IN_VERSION 9.1 MDR
RARE_DISEASE_URL http://rarediseases.info.nih.gov/gard/10308/scleroderma/resources/1 GARD
SID HP:0001594 HPO
SID HP:0007426 HPO
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
TERMUI T037309 MSH
TH UNK (19XX) MSH
TYPE_ID 900000000000013009 SNOMEDCT_US

Additional Concept Data:  (none)

URL to Bookmark: https://ncim-stage.nci.nih.gov/ncimbrowser/ConceptReport.jsp?dictionary=NCI Metathesaurus&code=C0011644

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