Introduction: Behçet's disease is a systemic inflammatory disorder characterized by vasculitis. Its typical features are recurrent oral and genital ulcerations with uveitis. Although vascular lesions are not listed among the criteria for diagnosis of Behçet's disease, up to 25-35% of the patients develop complications in arterial and venous large vessels.
Exegesis: We describe the case of a 45-year-old French man with Behçet-type vasculopathy. Though only one sign of Behçet's disease, i.e., oral ulcerations, was present, the patient had to undergo emergency surgery three times. The postoperative treatment combined corticosteroids, azathioprine, and oral anticoagulants. Three years later no therapy failure was observed.
Conclusion: On the basis of 1) recurrent aortic aneurysms, 2) large arterial and venous occlusive lesions, 3) superficial phlebitis, and 4) ulcerations of the aorta in macro- and microscopic examination of resected aortic walls, we concluded that the patient's life threatening vasculopathy was of the Behçet's type, even if several of the diagnostic features of Behçet's disease were lacking. Variations in clinical features of Behçet's disease are observed that might be due to hereditary traits, particularly to the genetic expression of an incomplete phenotype that would lead to the lack of typical clinical features.