Unusual right ventricular thrombus in a woman with Hughes-Stovin syndrome.

A 28-year-old woman with no prior pathologic antecedent was admitted to the internal medicine department for a 2month history of intermittent fever and swollen lower limbs. Physical examination showed bilateral pitting leg edema with no inflammatory skin changes. Her body temperature was above 38°C. Vascular echo-Doppler revealed a bilateral primitive iliac vein thrombosis extending to the vena cava. Echocardiography objectified a right ventricular rounded hyperechogen mass, suggesting myxoma or thrombus. Thoracic angio-CT-scan showed an aneurysm of the inferior branch of right pulmonary artery, surrounded by parenchymal ground-glass opacity in favour of vasculitis (Picture 1), and confirmed the diagnosis of right ventricular thrombus (Picture 2). Biologically, CRP was at 9 mg/L with no other laboratory assay abnormality. Thrombophilia screening and immunological tests were negative. HLA-B51 was positive. In the absence of suggestive manifestations (skin or ocular lesions) of Behcet’s disease (BD), the diagnosis of HughesStovin syndrome (HSS) was made. The patient was treated with bolus cyclophosphamide with heparin relayed by antivitamin K, under medical supervision. HSS is a rare entity characterized by the association of pulmonary artery aneurysms and peripheral venous thrombosis, which was first described in 1959 and classically affects men at a young age (1). The aetiology of HSS is still unknown; however it is thought to be a clinical variant manifestation of BD in which the typical symptoms such as oral