Acute aortic valve dysfunction in a woman with livedo racemosa and memory impairment

Subacute endocarditis Systemic lupus erythematosus Fibroelastoma Sneddon syndrome Answer: D Macroscopically, we found a deeply altered and thickened aortic valve with striking apposition of multiple, reddish thrombotic clusters (figure 1, upper sequence). Histological examination revealed massive tissue oedema without inflammatory elements and endothelial layer interrupted by stacks of fibrous tissue and dense adherent thrombi (figure 1, lower sequence). Sneddon syndrome (SS) is a non-inflammatory thrombotic arteriopathy that involves medium-sized and small-sized arteries, predominantly of dermal and cerebrovascular tissues, and affects mostly women in their fifth decade.1 SS is typically characterised by the combination of livedo racemosa and neurological symptoms, such as headaches, dizziness and recurrent transient ischaemic attacks, which lead to an early form of dementia.2 Thrombosis of heart valves occurs and anticoagulation therapy should be adopted.