Behçet-type vasculopathy in a patient without the diagnostic features of Behçet disease

✍ Scribed by Brian D. Golden; Archana Goel; Hal J. Mitnick

• Publisher: John Wiley and Sons
• Year: 1996
• Tongue: English
• Weight: 504 KB
• Volume: 39
• Category: Article
• ISSN: 0004-3591
• DOI: 10.1002/art.1780391122

✦ Synopsis

Beheet's disease is a multisystem inflammatory disorder which may involve the vascular system. The vasculopathy of Beheet's disease is distinctive among the vasculitides in that it involves both arteries and veins, and vessels of all sizes. Most published diagnostic criteria for Behget's disease include the classic triad of orogenital ulceration and ocular inflammation. In this report, we describe a patient who had a vasculopathy fitting the Behget's disease type, but who lacked the other characteristic or diagnostic features of Behget's disease. This case illustrates an unusual presentation and natural history of a complex vasculitic disease.

Behqet's disease is a systemic illness characterized by oral and genital ulceration and ocular inflammation (1). In patients with this triad, the disease may also involve the vascular, neurologic, or gastrointestinal systems (2). Herein we describe a patient who had a systemic vasculopathy that was highly characteristic of that seen in Behqet's disease, but who had none of the diagnostic features of the BehCet triad.

CASE REPORT

The patient, a previously healthy 39-year-old African-American man, presented to the hospital in February 1993 with a pulsatile mass in the left upper thigh. An angiogram revealed saccular aneurysms in 4 arteries: the left superficial femoral artery, left popliteal artery, and bilateral hypogastric arteries. The left superficial femoral artery aneurysm ruptured acutely and was resected in continuity with the left popliteal aneurysm and repaired using saphenous vein. Pathologic examination of these arteries revealed mucoid degeneration.