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

This study was undertaken to investigate the immunological mechanism of Behqet s syndrome, considered to be important in the pathogenesis of the disease. Seventy- three patients with complete, incomplete and suspected types of Behget's syndrotne were tested for leukocyte migration ingibition factor(LIF), one of the lymphokines. The results were as follows : 1. There was no difference between the average LIF activity of all the patients and that of eontrol. 2. LIF activity of complete type, according to Shirnizus classification, was significaritly lower than the control value. 3. LIF activity of ocular type, according to Lehners classification, was signficantly lower than the control value. 4. LIF activity for patients with 4 clinical symptoms was well below the value for patients with less symptomes 5. For patients with single clinical symptom, LIF activity of complete type was well below the values of incomplete and suspected types. 6. In suspected and mucocutaneous types, LIF activity was low when the patients showed two clinical symptoms than one. Thus, LIF activity was low for patients with complete, ocular and neurological types and with multiple symptorns.
Behcet Syndrome* ; Classification ; Humans ; Leukocytes* ; Lymphokines

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 disease occurs with a high prevalence in the Far East including Korea. In this report we inspected 1,155 patients with Behcet's disease and collected information concerning the clinical and epidemiologic features of Behcet's disease in Korea. In summary, patients in their 30s were the most common, with the most common age of onset in the 20s; the sex ratio was 0.63:1 with female predominance; according to revised Shimizu's classification, the order of frequency of the different types was incomplete (38.2%), suspected (25.4%), possible (20.3%) and complete (16.1%); oral ulcers were the most frequent major symptom (97.5%) and the most common initial symptom (78.5%); erythema nodosum-like lesion (55.3%) was the most common type of skin lesion; a seasonal aggravation of the disease was observed in 21.3% of patients; and the most common type of childhood onset Behcet's disease was suspected type.
Adolescence ; Adult ; Age Distribution ; Aged ; Behcet's Syndrome/epidemiology* ; Behcet's Syndrome/complications ; Behcet's Syndrome/classification ; Child ; Female ; Human ; Korea/epidemiology ; Male ; Middle Age ; Sex Distribution

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

Neurologic studies were done on 7 neuro-Behcet's syndrome patients, admitted from June 1985 to June 1986. They were from 30 to 43 years of age with mean age nf 35. 3;3 were male and 4 female. Symptoms fitting more than 3 of the 4 major diagnostic criteria included in Shimizu's classification preceded the neurological symptoms in all 7 patients and they progressed to neuro-Behcet syndrome approximately 5 years after the appearance of the first major syrnptoms of Behcet's syndrome, Severe headache was seen in all 7 patients. Ataxia, dysarthria, increased DTR, and herniplegia. were other rnajor signs found and psychiatric symptoms were present in 2 cases. The cases were neurologically classified as brain stem syndrome in 3 male patients, meningo-encephalitic syndrome in 1, and mixed type in 3. Characterletic laboratory findinge were lncreaeed mononuclear celle in CSF, peripheial blood leukocytosis, and decreased lymphocyte blastogenesis.
Ataxia ; Behcet Syndrome* ; Brain Stem ; Classification ; Dysarthria ; Female ; Headache ; Humans ; Leukocytosis ; Lymphocyte Activation ; Lymphocytes ; Male

The clinical features of cutaneous manifestations in 411 patients with Behcet's syndrome were studied. 302 patients (73.5%) had skin lesions. The frequency with which the following skin lesions were noted was, in decreasing order; erythema nodosum-like lesion, papulopustular eruption, erythema multiforme-like lesion, thrombophlebitis, ulcer and Sweet's syndrome-like lesion. More than two types of skin lesions were seen in 86 patients (28.5%), the combination of the skin lesions being in decreasing order, erythema nodosum-like lesion and papulopustular eruption; erythema multiforme-like lesion and papulopustular lesion; erythema nodosum-like lesion and thrombophlebitis. A skin pathergy test was performed on 245 patients, and a positive reaction was seen in 97 patients (39.6%). This study showed the high incidence, wide spectrum and importance of skin lesions as a major symptom in Behcet's syndrome.
Adolescent ; Adult ; Aged ; Behcet Syndrome/complications* ; Behcet Syndrome/epidemiology ; Child ; Female ; Human ; Male ; Middle Age ; Skin Diseases/classification ; Skin Diseases/epidemiology* ; Skin Diseases/etiology

Fifty-two Korean patients with Behcet's syndome were typed for HLA antigens. 52 apparently healthy Korean subjects were used as controls; 42 for HLA-A, B, C and all 52 for HLA-DR typing. HLA-B5 and DRw8 presented significantly high frequencies in all patiens. According to Shimizu's classification, HLA-B5 and DRw8 were significantly increased in the complete type; B5 in the incomplete type; DR3 in the suspected-possible type. According to Lehner's classification, HLA-DR3 was significantly increased in the neurological type; B5 in the ocular type; B5 in the ocular type. We confirmed the association of HLA-B5 with the severity of Beh et's syndrome. A relation might exist between DRw8, DR3 and Behcet's syndrome.
Adolescent ; Adult ; Behcet Syndrome/*immunology ; Female ; HLA Antigens/*analysis/classification ; Human ; Korea ; Male ; Middle Age ; Support, Non-U.S. Gov't

Due to similar manifestations of intestinal Beh?et's disease (BD) to those of other colitis such as Crohn's disease or intestinal tuberculosis, it is still challenging for gastroenterologist to accurately diagnose intestinal BD in patients with ileo-colonic ulcers. Moreover, no reliable diagnostic criteria for intestinal BD have been developed yet. Therefore, IBD Study Group of KASID was formulated to establish the guideline for the diagnosis of intestinal BD using a modified Delphi process. The novel diagnostic criteria for intestinal BD were developed based on two aspects; colonoscopic findings and extra-intestinal systemic manifestations, in which patients were categorized into 4 groups including definite, probable, suspected, and non-diagnostic for intestinal BD. Furthermore, Disease Activity Index for intestinal BD was developed through a Korean multicenter study. These diagnostic and disease activity guidelines will contribute to understand intestinal BD.
Behcet Syndrome/classification/*diagnosis ; Blood Chemical Analysis ; Colonic Diseases/*diagnosis ; Colonoscopy ; Diagnostic Imaging ; Hematologic Tests ; Humans ; Severity of Illness Index

For the purpose of epidemiologic study, the patients who visited Severance Hospital Behcet's Syndrome Specialty Clinic were grouped by Shimizu classification into the complete, incomplete, suspected types and evaluated. The results of the evaluation with respect to personal characteristics, geographic distribution, socioeconomic characteristics, family history, and clinical characteristics are as follows. 1. The incidence among the subgroups was incomplete type(46.3%), suspected type(36.7%), complete type(17.0%) and the male to female ratio was 0.60: 1, and the average age of onset was 29.1. years for males and 27.6 years for females. 2. The occupational distribution among males included office workers, 31.5%; drivers, 10.6% and among females was office workers, 6.7%; teachers, 3. 7 %; unemployed, 81.7%. 3. Among the presumed aggrevating factors affecting oral cavity, recurrent tonsillitis(20.8%) was most common, and premenstrual exacervation(12.8%) was most common. 4. The most common initial major manifestation was oral ulcer(80.6%), and the longest duration of major manifestations was oral ulcer(average 6.4 years), and the most frequent relapse of major manifestations was oral ulcer(average 14.6 times/year). 5. The average duration between the appearance of the first and second major manifestations was 5.2 years for complete type, 6.3 years for incomplete type, and 6.6 years for suspected type. 6. Combinations of major manifestations most commonly included oral ulcer, genital ulcers and skin lesions(65.3%) for incomplete type, and oral ulcer, skin lesion(54.8%) for suspected type.
Age of Onset ; Behcet Syndrome* ; Classification ; Epidemiologic Studies ; Family Characteristics ; Female ; Humans ; Incidence ; Male ; Mouth ; Oral Ulcer ; Recurrence ; Skin ; Ulcer