Behcet's syndrome is a multi-systemic and chronic disorder that affects many organs. It has been suggested that the diagnosis was based on the presence of the 'major' and 'minor' clinical criteria. When thromobophlebitis, arthritis, central nervous system or gastrointestinal lesions are also present. Behcet's syndrome will be thought to be present in the appropriate geographic area. We report a case of superior vena cava syndrome caused by Behcet's disease in a 40-year-old man with recurrent oral aphthous ulcers and skin rashes on the anterior chest wall. There were multiple thrombosis of the superior vena cava, innominate and subclavian veins. This patient also had a solitary cecal ulcer with an ileocecal fistula and downhill varix. The chest CT, veno-cavography, pulmonary angiography and colon study were taken and follow-up was performed.
Adult ; Behcet's Syndrome/diagnosis ; Behcet's Syndrome/complications* ; Cecal Diseases/complications ; Human ; Intestinal Fistula/complications ; Male ; Superior Vena Cava Syndrome/etiology* ; Superior Vena Cava Syndrome/diagnosis ; Ulcer/complications

Adult ; Behcet Syndrome ; complications ; diagnosis ; Blood Sedimentation ; C-Reactive Protein ; analysis ; Cardiovascular Diseases ; etiology ; Humans ; Male

Primary hepatic leiomyosarcoma is a very rare tumor of the liver and primary hepatic leiomyosarcoma with Behcet's disease has not been reported previously. Behcet's disease is a multisystem disorder presenting with recurrent oral and genital ulcerations as well as ocular lesions; it has rarely been reported in association with malignant disease. We report a case of primary hepatic leiomyosarcoma with intrahepatic and abdominal subcutaneous metastasis in a patient with Behcet's disease; this is the first report of these findings in Korea.
Abdominal Neoplasms/diagnosis/*secondary ; *Abdominal Wall ; Adult ; Behcet Syndrome/*complications ; Female ; Humans ; Leiomyosarcoma/*complications/diagnosis/*secondary ; Liver Neoplasms/*complications/diagnosis/*secondary ; *Subcutaneous Tissue

Behcet's disease is a chronic multisystemic disorder involving many organs and characterized by recurrent oral and genital ulcers and relapsing iritis. A case of BD with large vein thrombosis involving superior and inferior vena cava is presented. Large vein thrombosis in BD is not commonly developed and most commonly observed in the inferior or superior vena cava. A review of the literature emphasizes the rarity of the combined superior and inferior vena caval occlusion. Existence of extensive large vein occlusion in BD is associated with limited therapy and poor prognosis.
Adult ; Behcet's Syndrome/diagnosis ; Behcet's Syndrome/complications* ; Disease Progression ; Fatal Outcome ; Female ; Fibrinolytic Agents/therapeutic use ; Human ; Phlebography ; Thrombosis/etiology* ; Thrombosis/drug therapy ; Thrombosis/diagnosis ; Vena Cava, Inferior/radiography* ; Vena Cava, Superior/radiography* ; Substances: Fibrinolytic Agents

PURPOSE: Behcet's disease (BD) is a chronic inflammatory disease characterized by orogenital ulcers, skin and ocular lesions, in addition to articular, vascular, and neurologic symptoms. Carpal tunnel syndrome (CTS), can also occur in BD patients secondary to inflammation in the connective tissues, vessels, and tendons, as well as nerve involvement in BD itself. However, reports of patients who have CTS in BD are rare. The aim of this study was to evaluate the clinical characteristics of CTS in BD patients. MATERIALS AND METHODS: Retrospective analysis of the medical records of 1750 BD patients, and 14 (0.8%) BD patients who were diagnosed with CTS was performed at the BD Specialty Clinic of Severance Hospital. Patient demographics, disease activity/severity for both diseases, and the clinical characteristics of CTS in BD were recorded and analyzed. RESULTS: All 14 BD patients with CTS were women. Twelve patients (85.7%) were diagnosed with active BD. The CTS was mild in 8 patients (57.2%), moderate in 3 patients (21.4%), and severe in 3 patients (21.4%). Ten patients (71.4%) had BD prior to the diagnosis of CTS, and these 10 patients all had active BD. CONCLUSION: CTS can occur as a result of the inflammation associated with BD and can also be the presenting symptom of nerve involvement in BD. CTS can also develop as the initial symptom of BD. Therefore, a higher degree of suspicion should be maintained for CTS in patients with BD and vice versa; however, the exact relationship is uncertain.
Adult ; Age Distribution ; Aged ; Behcet Syndrome/complications/diagnosis/*epidemiology ; Carpal Tunnel Syndrome/diagnosis/*epidemiology ; Female ; Humans ; Inflammation ; Male ; Middle Aged ; Retrospective Studies ; Sex Distribution

Approximately 50 % of patients with scleritis are known to be associated with systemic connective tissue diseases or vasculitic diseases such as rheumatoid arthritis (RA), Wegener's granulomatosis, relapsing polychondritis, and systemic lupus erythematosus. The patients with scleritis in association with Behcet's disease (BD) have been rarely described in the literature. We report a 46-year-old female patient with BD who developed the nodular scleritis.
Anti-Inflammatory Agents, Non-Steroidal/therapeutic use ; Behcet's Syndrome/drug therapy ; Behcet's Syndrome/diagnosis* ; Behcet's Syndrome/complications* ; Case Report ; Cyclophosphamide/therapeutic use ; Drug Therapy, Combination ; Female ; Follow-Up Studies ; Human ; Middle Age ; Prednisolone/therapeutic use ; Scleritis/drug therapy ; Scleritis/diagnosis* ; Scleritis/complications*

Behcet's disease (BD) is a chronic inflammatory condition involving several organs, such as skin, mucous membrane, eye, joint, intestine, lung and central nervous system. Ankylosing spondylitis (AS) is a prototype of seronegative spondyloarthropathy, and a chronic systemic inflammatory disorder of the axial skeleton, mainly affecting the sacroiliac joint and spine. In the latter, systemic complications may develop in addition to joint involvement. The coexistence of BD and AS has been rarely reported in the literature. The inclusion of BD among seronegative spondyloarthritides and whether sacroiliitis (SI) develops in BD are still being debated. We describe a 28-year-old man who has fulfilled the diagnostic criteria for BD and AS as well.
Adult ; Angiography ; Anti-Inflammatory Agents/administration +ACY- dosage ; Behcet's Syndrome/drug therapy ; Behcet's Syndrome/diagnosis+ACo- ; Behcet's Syndrome/complications+ACo- ; Case Report ; Drug Therapy, Combination ; Human ; Magnetic Resonance Imaging ; Male ; Spondylitis, Ankylosing/drug therapy ; Spondylitis, Ankylosing/diagnosis+ACo- ; Spondylitis, Ankylosing/complications+ACo- ; Treatment Outcome

We report an unusual case of unilateral frosted branch angiitis associated with Behcet's disease, including a review of previously reported cases. A 39-year-old male with history of recurrent oral and genital ulcers presented with visual loss in his left eye. Fundus findings demonstrated occlusive retinal vasculitis resembling acute frosted branch angiitis. Laboratory examinations including viral markers revealed no abnormal findings except positive HLA-B51. The patient was treated with systemic steroid and cyclosporine. Six months after presentation, new oral ulcers and pseudofolliculitis appeared, and he was diagnosed with Behcet's disease following rheumatology consultation. During follow-up, there was no change in visual acuity of hand movement, and disc neovascularization developed even after complete panretinal photocoagulation. Ocular manifestations of Behcet's disease can present as unilateral frosted branch angiitis, and may consecutively involve in both eyes. Early immunosuppressive treatment is recommended.
Adult ; Behcet Syndrome/*complications/diagnosis ; Diagnosis, Differential ; Fluorescein Angiography ; Fundus Oculi ; Humans ; Magnetic Resonance Imaging ; Male ; Retinal Artery/*pathology ; Retinal Vasculitis/diagnosis/*etiology

Thrombosis is an important complication of central venous catheterization. Among the many intrinsic and extrinsic factors, the patient's medical disease can play a role in thrombogenesis. Behcet's disease (BD), classified as a vasculitis, is a multisystem disease involving the small blood vessels. It is often difficult to recognize and diagnose the disease. A 24-yr-old female patient showed massive central venous thrombosis which caused superior vena cava syndrome after subclavian vein catheterization. Twenty days after catheterization, the patient exhibited swelling of the face, neck, and both upper extremities. Despite thrombectomy and continuous anticoagulation therapy, her facial and upper extremity swelling reappeared and follow-up chest computed tomography (CT) showed the recurrent thrombosis in the same central veins previously affected. A diagnosis of BD was then made. Following steroid therapy, neither clinical symptoms nor CT findings suggestive of central venous thrombosis were observed during the subsequent 6-months of follow-up period. This case emphasizes that central venous catheterization in a patient with BD should be performed with great caution.
Adult ; Behcet's Syndrome/*complications ; Case Report ; Catheterization, Central Venous/*adverse effects ; Female ; Human ; Phlebography ; Superior Vena Cava Syndrome/diagnosis/*etiology ; Tomography, X-Ray Computed ; Vena Cava, Superior

Behcet's disease (BD) has been recognized as multi-systemic chronic vasculitic disorder of recurrent inflammation, characterized by the involvement of multiple organs and resulting in orogenital ulcers, uveitis, and skin lesions. Involvement of the central nervous system, vessels, and intestines in BD often leads to a poor prognosis. Digestive manifestations in BD have been reported in up to 1-60% of cases, although the rate varies in different countries. The most frequent extra-oral sites of gastrointestinal involvement are the ileocecal region and the colon. Gastric or esophageal involvement is reported to be very rare. Moreover, there have been no reports on the simultaneous involvement of the esophagus, stomach, ileum, and colon. Here, we present a 55-year-old Korean man with intestinal BD and multiple ileal and colonic ulcerations complicated by perforation, gastric ulcer with bleeding followed by perforation, and esophageal ulcers with bleeding.
Behcet Syndrome/complications/*diagnosis/pathology ; Cecal Diseases/complications/pathology ; Diagnosis, Differential ; Endoscopy, Digestive System ; Gastrointestinal Diseases/complications/*diagnosis ; Gastrointestinal Hemorrhage ; Humans ; Intestinal Perforation/*diagnosis/etiology/pathology ; Male ; Middle Aged ; Peptic Ulcer Perforation/pathology ; Stomach Ulcer/complications/pathology