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Concept information

Preferred term

Sneddon Syndrome  

Type

  • Topical Descriptor

Entry terms

  • Livedo Reticularis And Cerebrovascular Accidents
  • Livedo Reticularis, Systemic Involvement
  • Sneddon Champion Syndrome
  • Sneddon-Champion Syndrome
  • Syndrome, Sneddon-Champion

Note

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

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.

History note

  • 1996

In other languages

URI

http://www.yso.fi/onto/mesh/D018860

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