- Concept UI
- M0028214
- Scope Note
- A systemic non-inflammatory arteriopathy primarily of middle-aged females characterized by the association of LIVEDO RETICULARIS, multiple thrombotic CEREBRAL INFARCTION; CORONARY DISEASE, and HYPERTENSION. Elevation of antiphospholipid antibody titers (see also ANTIPHOSPHOLIPID SYNDROME), cardiac valvulopathy, ISCHEMIC ATTACK, TRANSIENT; SEIZURES; DEMENTIA; and chronic ischemia of the extremities may also occur. Pathologic examination of affected arteries reveals non-inflammatory adventitial fibrosis, thrombosis, and changes in the media (From Jablonski, Dictionary of Syndromes & Eponymic Diseases, 2d ed; Adams et al., Principles of Neurology, 6th ed, p861; Arch Neurol 1997 Jan;54(1):53-60). Mutations in the CECR1 gene (ADA2 protein, human) are associated with Sneddon syndrome.
- Terms
-
Sneddon Syndrome
Preferred Term
Term UI
T056213
Date01/01/1999
LexicalTag
EPO
ThesaurusID
-
Livedo Reticularis, Systemic Involvement
Term UI
T370325
Date11/03/1999
LexicalTag
NON
ThesaurusID
NLM (2000)
-
Sneddon-Champion Syndrome
Term UI
T056212
Date07/12/1994
LexicalTag
EPO
ThesaurusID
NLM (1996)
-
Livedo Reticularis And Cerebrovascular Accidents
Term UI
T812296
Date11/15/2011
LexicalTag
NON
ThesaurusID
OMIM (2013)