A case of Hughes-Stovin syndrome (incomplete Behçet's disease) with extensive arterial involvement : Unmasking the true face of a rare syndrome.

Hughes-Stovin syndrome (HSS), characterized by the combination of multiple pulmonary artery aneurysms and deep vein thrombosis, is a rare and an under-recognized clinical entity with less than 40 published cases in English medical literature. Vascular venous thrombotic events, as occurring in the course of Behçet's disease (BD), are also described in HSS, e. g.

Hughes-Stovin syndrome: is it incomplete Behçet's? Report of two cases and review of the literature.

In this report, we described two male patients with Hughes-Stovin syndrome. The first patient was a 26-year-old male who initially presented with deep vein thrombosis (DVT) in the right lower limb followed shortly by hemoptysis. Pulmonary CT angiography revealed bilateral pulmonary artery aneurysms secondary to underlying pulmonary vasculitis.