A Case of Hughes - Stovin Syndrome.

Kim, Joo In; Lee, Young Min; Yum, Ho Kee; Choi, Soo Jeon; Choi, Seok Jin; Yang, Young Il; Sung, Kyu Bo; Kim, Dong Soon; Lee, Bong Choon

doi:1996.43.1.96

Tuberc Respir Dis > Volume 43(1); 1996 > Article

Case Report

Tuberculosis and Respiratory Diseases 1996;43(1):96-101.
DOI: https://doi.org/10.4046/trd.1996.43.1.96 Published online February 1, 1996.

A Case of Hughes - Stovin Syndrome.

Joo In Kim, Young Min Lee, Ho Kee Yum, Soo Jeon Choi, Seok Jin Choi, Young Il Yang, Kyu Bo Sung, Dong Soon Kim, Bong Choon Lee

¹Department of Internal Medicine, Inje University College of Medicine, Pusan, Korea.
²Department of Radiology, Inje University College of Medicine, Pusan, Korea.
³Department of Pathology, Inje University College of Medicine, Pusan, Korea.
⁴Department of Radiology, Asan Medical Center, Ulsan University, Seoul, Korea.
⁵Department of Internal Medicine, Asan Medical Center, Ulsan University, Seoul, Korea.

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.

Key Words: Hughes-stovin syndrome, Pulmonary artery aneurysm, Deep vein thrombosis, Embolization therapy