上矢状洞血栓症を伴つたVasculo-Behçet病の1症例

VASCULO-BEHÇET'S DISEASE WITH SUPERIOR SAGITTAL SINUS THROMBOSIS:CASE REPORT

　抄録　上矢状洞血栓症を伴つたVasculo-Behçet病の1症例を報告した。症例は30歳男性。学童期より口腔内アフタが反復して出現。数年前から陰部潰瘍，湿疹，結節性紅斑が多発し，頭痛，視力障害を主訴として来院。入院時，左下肢に有痛性の腫脹，色素沈着，結節性紅斑を認め，神経学的には両側の著明なうつ血乳頭を認めた。血管撮影にて上矢状洞に多発性の閉塞を認め，左膝窩動脈および左大腿静脈群にも閉塞を認めた．臨床症状，組織学的所見，血清免疫学的検査によりVasculo-Behçet病と診断した。頭蓋内静脈洞に血栓を伴つたBehçet病の報告は過去に4例みられるのみで，上矢状洞血栓症の合併は調べ得た範囲では未だ報告はなかつた。従来，pseudotumor cerebriやsinus thrombosisと診断されていた疾患群には，本症例のごとく全身性系統的疾患の一部分症であることが，稀ながらあることを示した。

The patient is a 30-year-old man who has suffered from recurrent attacks of tonsilitis, oral aphthae and scrotal ulcerations, erythema nodosum and thrombophlebitis. In April, 1980, he gradually developed headache and visual disturbance. On Ap-ril 14, 1980, he was pointed out remarked bilateral choked disc by an ophthalmologist and then admit-ted to the Miyazaki Medical College Hospital. On admission to our service, he showed atypical symp-toms of Behçet's disease, namely, oral aphthae and scrotal ulcerations, erythema nodosum and bilateral choked disc. Laboratory data demonstrated hyper-immunoglobulinemia, increased clotting factors and decreased fibrinolytic activity. Immunogenetically, HLA BW51 type was demonstrated. The angio-grams showed complete obstructions of the superiorsagittal sinus and the common trunk of the femoral artery. Histological examination of the skin lesion demonstrated atypical chronic inflammation and thrombophlebitis. A diagnosis of atypical Vasculo-Behçet's disease was made. The response to the steroid therapy was dramatic, though the fibrinoly-tic drugs, anticoagulants and vasodilators were not effective. Thrombophlebitis is a well recognized complication of Behçet's disease occuring in major vessels, however thrombosis of the dural sinus has rarely reported. This case may be the first one which had superior sagittal sinus thrombosis with Vasculo-Behçet's disease in literature. We discussed the mechanism of the thrombogenesis, the relati-onship to HLA, the coexistence of Neuro-Behçet's disease and the therapy of Vasculo-Behçet's disease.