A Familial Syndrome of Arterial Dissections with Lentiginosis

Abstract

Arterial dissection occurs when blood enters a vessel wall through an intimal tear and a false lumen of blood is formed within the media.1,2The artery most commonly affected by dissection is the aorta,1followed by the renal3,4and extracranial internal carotid2,5arteries. 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—9and their association with various heritable connective-tissue disorders.10,11In our experience with cervical-artery dissections,. . . © 1995, Massachusetts Medical Society. All rights reserved.