Vasculo–Behçet's syndrome with widespread arterial involvement

Abstract

An 18-year-old woman with a history of multiple oral ulcers followed by erythema nodosum was admitted to our hospital because of the lack of a pulse in her upper left extremity and occasional dizziness. High C-reactive protein (CRP) levels were detected in her serum. Arterial angiography showed a widespread narrowing of the major arteries, including both carotid arteries, the left vertebral artery, the left subclavian artery (branches of the aortic arch), the abdominal aorta, and left renal arteries. However, no involvement in the circle of Willis was noted, and this was confirmed by magnetic resonance angiography. Pulmonary scintigraphy showed no perfusion defect. The distribution of the arterial involvement, her youth, and nega-tive human leukocyte antigen (HLA) B51 were consistent with Takayasu arteritis, although the presence of mucocutaneous involvement favored a diagnosis of vasculo-Behçet's syndrome. We treated the patient with prednisolone and warfarin, which resulted in an improvement in CRP levels and no thrombosis-related complications.