Loiseau-Corvez M N, de Kerdanet M M, Chevrant-Breton J, Le Gall E, Jezequel C
Service de Pédiatrie A, Hôpital-Sud, CHR Rennes.
Arch Fr Pediatr. 1992 Nov;49(9):815-7.
Behçet's disease is rare in children and is exceptionally revealed by thrombophlebitis at this age of life.
A girl, aged 13 years, was admitted after having suffered from thrombophlebitis of the right leg for 6 weeks. Her medical history included frequent attacks of aphthous stomatitis and conjunctivitis and one episode of iritis. At admission, thrombophlebitis with venous thrombosis was confirmed by Doppler; it was associated with aphthous gingivitis and two similar erosive lesions in the genital area. Technetium 99 perfusion scintiphotography revealed a right pulmonary embolism. The erythrocyte sedimentation rate was elevated but there was no change in coagulation factors or immunologic abnormalities, except for the presence of immune complexes and increased complement C3 component. Biopsy of the skin lesions showed vascularitis. There was no involvement of the eyes. The condition progressively improved with anticoagulant treatment for 1 year, followed by low doses of acetylsalicylic acid.
Phlebothrombosis, a classic complication of Behçet's disease in adults, revealed the disease in this girl. Its mechanism remains unclear and duration of anticoagulant treatment is still debated.
白塞病在儿童中罕见，在这个年龄段极少以血栓性静脉炎为首发表现。
一名13岁女孩，因右下肢血栓性静脉炎6周后入院。她的病史包括复发性阿弗他口炎和结膜炎发作频繁，以及一次虹膜炎发作。入院时，多普勒检查证实为伴有静脉血栓形成的血栓性静脉炎；伴有阿弗他牙龈炎和生殖器区域两处类似的糜烂性病变。锝99灌注闪烁照相术显示右肺栓塞。红细胞沉降率升高，但凝血因子无变化，免疫学检查除存在免疫复合物和补体C3成分增加外无异常。皮肤病变活检显示血管炎。眼部未受累。经抗凝治疗1年，随后服用低剂量乙酰水杨酸，病情逐渐好转。
静脉血栓形成是成人白塞病的经典并发症，在该女孩中揭示了此病。其机制仍不清楚，抗凝治疗的持续时间仍存在争议。
