Oh S-H, Lee J-H, Shin J-U, Bang D
Department of Dermatology and Cutaneous Biology Research Institute, Yonsei University College of Medicine, 134 Shinchon-dong, Seodaemun-gu, Seoul 120-752, Korea.
Br J Dermatol. 2008 Sep;159(3):555-60. doi: 10.1111/j.1365-2133.2008.08679.x. Epub 2008 Jun 29.
Behçet disease (BD) is a multisystemic disorder that is classified as vasculitis and can affect all types and sizes of blood vessels. Although vascular diseases are not regarded as essential for a diagnosis of BD, vascular complications can be fatal.
The purpose of this study was to examine the clinical characteristics of 14 patients with BD accompanied by an obstruction of the superior vena cava (VC), the inferior VC, or both, and then determine the dermatological features that indirectly suggest VC obstructions in patients with BD.
Among the 3500 patients registered at the BD Specialty Clinic of Severance Hospital from 1997 to 2006, 14 patients were diagnosed with BD-associated VC obstructions. Their medical records were reviewed.
Nine of the 14 patients with BD were male, and the age range for disease onset was 11-46 years. Computed tomography revealed superior VC thrombosis in seven patients, inferior VC thrombosis in four patients, and the involvement of both in three patients. The most prominent features in superior VC obstruction were facial oedema (n = 7, 50%) and neck vein distension (n = 6, 43%). Other features included dilated veins in the chest wall, upper body oedema, proptosis, and upper extremity swelling. Patients with an inferior VC obstruction commonly had dilated veins on the abdominal walls. These patients also had abdominal distension and lower extremity swelling. Corticosteroids, colchicine, immunosuppressants, and anticoagulants with or without heparinization were the most common treatment regimens giving good response. Attempts to treat patients with a high dose of steroid pulse therapy were ineffective. Ballooning venoplasty was a useful therapeutic modality for VC thrombosis during the early stage, although this procedure could not be performed in patients with BD with severe thrombosis.
Several cutaneous features of VC obstruction should be recognized in patients with BD. Early detection might permit an early diagnosis and treatment of occlusion of the VC.
白塞病（BD）是一种多系统疾病，归类为血管炎，可影响所有类型和大小的血管。虽然血管疾病并非BD诊断的必要条件，但血管并发症可能是致命的。
本研究旨在检查14例伴有上腔静脉（VC）、下腔静脉或两者阻塞的BD患者的临床特征，然后确定间接提示BD患者VC阻塞的皮肤特征。
在1997年至2006年在Severance医院BD专科门诊登记的3500例患者中，14例被诊断为BD相关的VC阻塞。回顾了他们的病历。
14例BD患者中9例为男性，发病年龄范围为11 - 46岁。计算机断层扫描显示7例患者有上腔静脉血栓形成，4例患者有下腔静脉血栓形成，3例患者两者均受累。上腔静脉阻塞最突出的特征是面部水肿（n = 7，50%）和颈静脉扩张（n = 6，43%）。其他特征包括胸壁静脉扩张、上身水肿、眼球突出和上肢肿胀。下腔静脉阻塞的患者通常腹壁静脉扩张。这些患者还伴有腹胀和下肢肿胀。皮质类固醇、秋水仙碱、免疫抑制剂和抗凝剂（无论是否肝素化）是最常见的治疗方案，反应良好。尝试用大剂量类固醇脉冲疗法治疗患者无效。球囊血管成形术是早期VC血栓形成的一种有用治疗方式，尽管在严重血栓形成的BD患者中无法进行此操作。
BD患者应认识到VC阻塞的几种皮肤特征。早期发现可能有助于早期诊断和治疗VC阻塞。
