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.

Annotation

do not confuse with SNEDDON-WILKINSON DISEASE see SKIN DISEASES, VESICULOBULLOUS

DUI

D018860 MeSH Browser

CUI

M0028214

Previous indexing

Cerebrovascular Disorders (1983-1995); Skin (1979-1983); Skin Diseases, Vascular (1993-1995)

History note

1996

Public note

1996