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Case Reports in Medicine
Volume 2013, Article ID 740837, 12 pages
Case Report

Behçet’s Disease, Associated Large Vessel Thrombosis, and Coexistent Thrombophilia: A Distinct Nosological Entity?

1st Department of Internal Medicine, “Georgios Papanikolaou” General Hospital, Exochi, 570 10 Thessaloniki, Greece

Received 15 February 2013; Revised 22 May 2013; Accepted 10 June 2013

Academic Editor: Christos D. Lionis

Copyright © 2013 Dimitrios Stoimenis et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Behçet’s disease (BD) represents a multisystemic disorder that combines features of immune-mediated diseases and autoinflammatory disorders. Even though it is recognized that every type or size of vessel can be affected in this disease, there is an inability to describe a coherent model that sufficiently explains the predilection of certain patients with BD for manifesting severe large vessel thrombosis. The inconsistent epidemiologic data and the complex genetic background of BD, along with the controversy of multiple international studies regarding the coexistence of thrombophilia in patients with BD and large vessel thrombosis, make us think that a percentage of these patients may actually suffer from a distinct clinical entity. The stimulus for this concept arose from the clinical observation of three male patients who were admitted to our clinic due to extended vena cava thrombosis. On the occasion of those clinically and laboratory resembling cases, we performed a literature review concerning the epidemiology of BD, associated thrombosis, and coexistent thrombophilic factors, in order to present some evidence, which sustains our hypothesis that certain patients with large vessel thrombosis, who share features of BD and coexistent thrombophilia, should actually be further investigated for the possibility of suffering from a distinct nosological entity.