광범위한 심부 혈전증 및 Budd - Chiari 증후군을 동반하여 급성 간 부전으로 사망한 Behcet 씨병 1 예

Behcet`s disease is a systemic disease, which can invlove any part of the body mainly affecting young adult. The pathogenesis of Behcet`s disease is not well known. Cardiovascular involvement is rare but may be fatal complications of Behcet`s disease. It is manifested by SVC or IVC thrombosis, extensive peripheral venous thrombosis or arterial aneurysm etc. We experienced a fatal case of Behcet`s disease with progressive, extensive venous thrombosis which resulted in Budd-Chiari syndrome and acute liver failure. In this case, we didn`t find any other usual clinical manifestation of Behcet`s disease such as aphthous oral ulcer, skin lesion, or other activity indices, during period of rapidly progressive deep vein thrombosis and development of Budd-Chiari syndrome. Deep vein thrombosis can be a fatal complication of Behcet`s disease so that early detection and proper treatment to prevent progressive thrombosis should be emphasized for longterm care of Behqet`s disease.