A Familial Syndrome of Arterial Dissections with Lentiginosis

Arterial dissection occurs when blood enters a vessel wall through an intimal tear and a false lumen of blood is formed within the media.1,2 The artery most commonly affected by dissection is the aorta,1 followed by the renal3,4 and extracranial internal carotid2,5 arteries. The pathogenesis of arterial dissection is incompletely understood, but a primary arteriopathy is frequently suspected, especially in younger patients. The possibility of a genetic predisposition to such an arteriopathy is supported by the familial occurrence of arterial dissections6–9 and their association with various heritable connective-tissue disorders.10,11 In our experience with cervical-artery dissections, . . .