A Case of Hughes - Stovin Syndrome / 결핵

ABSTRACT

Hughes-Stovin Syndrome is an exceedingly rare combination of distal pulmonary arterial aneurysm and deep vein thrombosis, mostly found in young patients. There are striking similarities between the vascular manifestation of Behcet's disease and Hughes-Stovin Syndrome. It has been suggested that they may have a similar pathogenesis. Most patients died of massive hemoptysis due to rupture of aneurysm. Recently we have experienced the first case of Hughes-Stovin Syndrome in Korea. A 37 year old male patient was admitted because of recurrent hemoptysis and intermittent fever. He had a history of recurrent aphthous ulcers and erythema nodosum-like skin rash, But no other findings of Behcet's disease was found. Angiography showed multiple pulmonary arterial aneurysm and deep vein thrombosis in Right lower extremity. Histologic examination of specimens of open lung biopsy revealed leukocyto- clastic angiitis. Pulmonary arterial aneurysms were successfully treated by coil embolization and he is in good condition with corticosteroid and cyclophosphamide therapy.