Hughes-Stovin syndrome revealing the presence of Behçet’s Disease

BACKGROUND: Hughes-Stovin Syndrome (HSS) is a rare clinical disorder characterized by deep venous thrombosis and multiple pulmonary and/or bronchial aneurysms. Aneurysms in systemic circulation can also be seen. CASE PRESENTATION: We report the first case of HSS with aortic aneurysm in a 55-year-old man who initially presented with deep venous thrombosis. The diagnosis of HSS revealing Behçet’s disease was made given the history of recurrent oral and genital ulcers. Treatment consisted of 3 daily pulses of methylprednisolone (1g) followed by oral prednisone (1mg/kg daily) and 6 monthly pulses of cyclophosphamide. Oral anticoagulation treatment was held 3 months and then was stopped with good outcome. CONCLUSION: Systemic aneurysms in Hughes Stovin is a worth knowing complication which may reveal Behçet’s disease.