There are no specific diagnostic laboratory tests for Behcet's disease. Diagnosis relies on proper history-taking and typical clinical manifestations. To provide more objectivity to the diagnosis, several diagnostic criteria have been introduced including one major set of guidelines by the International Study Group for Behcet's Disease (ISGBD) in 1990 which has made a significant contribution, although some disagreements exist in interpretation. Based on this criteria, recurrent oral ulceration is an obligatory manifestation for the diagnosis of Behcet's disease; however, some data indicates it is not a requirement for the diagnosis. In this article the author critically reviews different accepted diagnostic criteria or classifications of Behcet's disease and gives recommendations for a more thorough diagnosis.
Behcet's Syndrome/diagnosis* ; Behcet's Syndrome/classification ; Human

PURPOSE: Behcet's disease (BD) is a chronic, relapsing, multi-system vasculitis of unknown aetiology with complicated and diversified clinical features predominantly involving oral and genital ulcers, and ocular and cutaneous lesions. The clinical features of this disease have been described to be different according to geographical areas and gender. We investigated the specific clinical features of BD patients in Northeastern China. MATERIALS AND METHODS: 116 patients involved in this study fulfilled the classification criteria of the International Study Group for BD. The clinical manifestations and results of laboratory tests of BD were recorded in each patient. RESULTS: The onset was typically between 20-39 years with a slight female predominance. Oral ulcers were the most common manifestation, followed by skin lesions, positive pathergy reaction/genital ulcers, and ocular lesions. Vascular lesion and epididymitis were rare in patients with BD. The frequency of erythema nodosum-like lesion and articular involvement were significantly higher in females, while gastrointestinal involvement was significantly higher in males. The results of laboratory tests showed that the human leukocyte antigen (HLA)-B*51 alleles were positive in 30.9% of patients and the immunological abnormities were present in some patients. CONCLUSION: The clinical features of BD showed geographical and gender difference. Genetic and immune factors might participate in aetiopathogenesis of BD.
Behcet Syndrome/*diagnosis/epidemiology/pathology ; China ; Female ; Geography ; Humans ; Male

A 30 year-old woman with a history of Behcet's disease was admitted to our clinic because of a paroxysmal attack of palpitations, headache, and ocular pain. Initially, we did not suspect a pheochromocytoma. We evaluated Behcet's disease activity because the cardiovascular and neurological symptoms mimicked those of Behcet's disease. Pheochromocytoma is often overlooked and can be fatal if not recognized and treated appropriately. In the present case, abdominal computerized tomography incidentally revealed a mass confirmed to be a pheochromocytoma in the left adrenal gland, and the tumor was successfully removed. This is the first Korean report of a pheochromocytoma in a patient with Behcet's disease. We suggest that pheochromocytoma should be included in the differential diagnosis of Behcet's disease if a patient presents with cardiovascular or neurological symptoms.
Adrenal Glands ; Behcet Syndrome ; Diagnosis, Differential ; Female ; Headache ; Humans ; Pheochromocytoma

The criteria employed for Korean Behcet's disease (BD) from January 1990 to December 2000 have been investigated, and the diagnostic validity for those criteria was determined. For the generation of a modified set of preliminary criteria from the International Study Group (ISG) criteria, the diagnostic values for individual feature of BD were calculated. The criteria by the Behcet's Disease Research Committee of Japan appeared to be widely employed with the ISG criteria. However, because the ISG criteria revealed a relatively valid outcome in Korea, the application of this criteria will be needed for the universal unification until the criteria with better performance comes out. On the other hand, the modified set of preliminary criteria that consisted of the clinical items with better results seemed to improve some pitfalls of the ISG criteria. Although that criteria showed better performance than the preexisting criteria, it should be necessary to validate its effectiveness in other areas.
Behcet Syndrome/classification ; Behcet Syndrome/diagnosis* ; Behcet Syndrome/epidemiology ; Bibliometrics ; Data Collection ; Databases, Factual ; Human ; Korea/epidemiology ; Likelihood Functions ; Prevalence ; Sensitivity and Specificity

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

Recurrent aphthous stomatits (RAS) is also known as recurrent oral ulcers, recurrent aphthous ulcers, or simple or complex aphthosis. RAS is the most common inflammatory ulcerative condition of the oral mucosa in North American patients. RAS has been the subject of active investigation along multiple lines of research including epidemiology, immunology, clinical correlations and therapy. Clinical evaluation of the patient requires correct diagnosis of RAS and classification of the disease based on morphology (MIAU, MJAU, HU) and severity (simple versus complex). In order to properly diagnose and treat a patient with lesions of RAS, the clinician must exclude other causes of acute oral ulcers. Complex aphthosis and complex aphthosis variants associated with systemic disorders should be considered. The aphthous-like oral ulcerations of patients with HIV disease represent a challenging differential diagnosis. The association of lesions of RAS with hematinic deficiencies and gastrointestinal diseases provides an opportunity to identify a "correctable cause" which, with appropriate treatment, can result in a remission or substantial lessening of disease activity. Finally, when all of these factors are considered, the evaluation of the patient for Behcet's disease can be continued on firm grounds that one of the major criteria for the diagnosis of Behcet's disease has been met.
Behcet's Syndrome/diagnosis* ; Diagnosis, Differential ; Human ; Recurrence ; Stomatitis, Aphthous/etiology ; Stomatitis, Aphthous/diagnosis* ; Stomatitis, Aphthous/classification

Behcet disease is relatively rare in pediatric age group. And the bowel involvement is seen in only a small portion of Behcet disease. However, once the bowel is involved it is potentially life threatening event. We report a 15 year old boy with intestinal Behcet's disease who had a history of recurrent oral and genital ulcers for several years. He underwent right hemicolectomy under the impression of intestinal tumor. Pathologically the lesion was a large sharply delineated ulcer in the cecum. The ulcer was round and deep with elevating margin, and was associated with thickening of affected intestinal wall. Microscopically, the ulcer base consisted of granulation tissue with fissurings and underminings. Characteristic phlebitis and occlusive arterial lesion were seen in intestinal wall. The inflammatory lesion was most pronounced around the ulcer but could be recognizable throughout the resected specimen.
Adolescent ; Behcet Syndrome/*diagnosis/pathology ; Diagnosis, Differential ; Humans ; Intestinal Diseases/*diagnosis/pathology ; Male ; Ulcer/pathology

Intestinal Behçet's disease (BD), generally accepted as a type of inflammatory bowel disease (IBD), could be diagnosed when patients with BD have objectively documented gastrointestinal symptoms and intestinal ulcerations. Similar to IBD, intestinal BD has an unpredictable disease course with relapse and remission and is often related to a poor prognosis. However, there is no single gold standard for assessment of the disease activity of intestinal BD, and its diagnosis and management depend heavily on expert opinions. The Korean IBD Study Group recently developed novel diagnostic criteria based on colonoscopy findings and clinical manifestations using a modified Delphi process to overcome drawbacks of previously used consensus for the diagnosis of intestinal BD. In addition, the recently developed disease activity index for intestinal BD, consisting of a relatively simple 8-point index, could also help in determining treatment strategies and monitoring therapeutic responses. In this review, the progress in the diagnosis and disease activity measurement of intestinal BD will be discussed.
Behcet Syndrome ; Colonoscopy ; Consensus ; Diagnosis* ; Expert Testimony ; Humans ; Inflammatory Bowel Diseases ; Prognosis ; Recurrence ; Ulcer

Subarachnoid hemorrhage due to a solitary spinal aneurysm is extremely rare, and diagnosis and treatment are challenging. We report a rare case of a ruptured radiculomedullary artery aneurysm in a patient with Behçet disease. A 49-year-old man presented with severe lower abdominal and leg pain. Magnetic resonance imaging was performed and an enhanced intradural-extramedullary lesion at the T12 spinal level with subarachnoid hemorrhage was identified. Diagnostic spinal angiography was performed to evaluate the vascular lesion, and a radiculomedullary artery aneurysm at the T12 level was identified. We performed surgical resection of the aneurysm and a good neurological outcome was obtained.
Aneurysm* ; Angiography ; Arteries* ; Behcet Syndrome ; Diagnosis ; Humans ; Leg ; Magnetic Resonance Imaging ; Middle Aged ; Subarachnoid Hemorrhage

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