Dermatological features in Behçet disease-associated vena cava obstruction

Background: 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. Objectives: 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. Methods: 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. Results: 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. Conclusions: 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.