Sneddon's syndrome

Arteriopathy affecting the brain and skin (livedo reticularis). The combination was previously described (before Sneddon's 1965 paper) in patients with endarteritis obliterans and syphilis. The diagnosis mandates EXCLUDING a recognized infectious, inflammatory, or connective tissue disease.

Livedo reticularis is arterial constriction due to a progressive obliterative process due to cutis marmorata, whereas livedo racemosa is a netlike skin pattern is a response to cold that persists on warming. LR has many associations besides Sneddon's syndrome. Stroke may occur decades after or even before LR with average age 42. Stroke can occur in MCA, TIA, seizures, cognitive impairment, TMB, and others. Association of Sneddon's syndrome include labile hypertension, ischemic heart disease, mitral valve thickening, aPL's and miscarriages. Primary antiphospholipid syndrome, has , in addition to the above, common thrombocytopenia, venous thrombosis, but unlike Sneddon's, not LR or cerebral ischemia.

On MRI in Sneddon's see single or multiple small or medium sized infarcts and angio shows occlusions of multiple distal branches. Angio of hands also shows multiple arterial occlusions. Path, Sneddon's is an arteriopathy of small and medium sized vessels although the skin biopsies may be negative. The role of APL's and genetics is probable but not clear. Coumadin is preferred if cardiac valvular disease exists or venous thrombosis.