Vascular Behcet syndrome without definite Behcet's disease.

Introduction. - Behcet's disease is a systemic inflammatory disorder charac terized by vasculitis. Ifs typical features are recurrent oral and genital ulcerations with uveitis. Although vascular lesions are not listed among th e criteria for diagnosis of Behcet'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 Behcet-ty pe vasculopathy: Though only one sign of Behcet's disease, i.e., oral ulcer ations, was present, the patient had to undergo emergency surgery three tim es. 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 arter ial and venous occlusive lesions, 3) superficial phlebitis, and 4) ulcerati ons of the aorta in macro- and microscopic examination of resected aortic w alls, we concluded that the patient's life threatening vasculopathy was of the Behcet's type, even if several of the diagnostic features of Behcet's d isease were lacking. Variations in clinical features of Behcet's disease ar e observed that might be due to hereditary traits, particularly to the gene tic expression of an incomplete phenotype that would lead to the lack of ty pical clinical features. (C) 2000 Editions scientifiques et medicales Elsev ier SAS.