肺梗塞を契機に発見された血管型Behçet病の1例

A Case of Vasculo:Behçet's Disease with Pulmonary Infarction

　血管病変を初発症状とし，特異な経過で発症したBehçet病の1例を報告する．症例は17歳，男性．11歳頃から口腔内アフタを反復していたが放置．1992年4月より両側下腿腫脹と，下腿伸側に反復する皮疹が出現．1993年5月より毎朝少量の血痰が出現し，改善を示さないため近医を受診した．胸部X線写真上，左下肺野に実質性の異常陰影を指摘され，1993年11月26日精査目的にて当院入院．臨床経過と肺血流スキャン所見から肺梗塞と診断した．下腿腫脹の原因検索のため静脈造影を施行し，下大静脈閉塞と深部静脈血栓症を認めたが，血管内視鏡では下大静脈閉塞部に血栓は認めなかった．入院後に虹彩炎，毛嚢炎様皮疹，口腔内アフタが出現し，陰嚢潰瘍の既往からBehçet病と診断した．肺動脈造影で左下葉枝に血管炎が疑われ，肺梗塞の誘因となる血栓は下大静脈よりむしろ肺動脈に由来すると考えられた．

A 17 years-old male had complained of hemosputumfor 6 months. He had had recurrent episodes of oral ulcers since he was 11 years old. He also had been operated on for hemorrhoids at 12 and had presented bilateral lower limb swelling and recurrent skin rash since the age of 14. At 16 he presented genital ulcera-tion. Chest X-ray films showed a round opacity in the left lower lung fields. The pulmonary scintigraphy revealed a defect in the left lower lobe. The pulmonary angiography demonstrated several areas of arterial stenosis and occlusion of the left pulmonary artery branches. The venogram demonstrated many collateral vessels in both lower extremities and obstruction of the supeior vena cava. The lumen of the superior vena cava was observed by the angiofiberscopy that showed adhe-sion of the vessel wall at the point of obstructionwithout any thrombus.

During hospitalization, oral ulcer, folliculitis and uveitis appeared. From these findings he was then diagnosed as having vasculo-Behçet's disease. This case had the peculiar presentation of throboembolic symptoms preceding the major features of Behçet's disease. We supposed that, in this case, the origin of the thrombus that provoked the peripheral thromboembolic episodes was in the pulmonary arteries. This is a very rare case of complete vasculo-Behçet's disease with pulmonary infarction.