Loss of vision in Behcet's disease is one of the most common, as well as one of the most serious, of its varied manifestations. Total blindness may often be the eventual outcome. The underlying problem in the eye, as well as in other organ systems, is an occlusive vasculitis. Although the most common ocular symptom is that of iridocyclitis, the presence of retinovascular lesions, especially necrotizing retinitis, is well known but often obscured by the severity of the anterior reaction, which precludes a good view of the retina. While Behcet's disease is characterized by spontaneous remissions and relapses, no external factors have been found to explain the length of remissions. Blindness, which occurs in up to 25% of patients, is one of the major causes of permanent disability.
Behcet's Syndrome/therapy ; Behcet's Syndrome/pathology* ; Eye/pathology* ; Human ; Prognosis

Dermatologic lesions in Behcet's disease are regarded as important manifestation for diagnosis. Various kinds of cutaneous lesions appear in patients with Behcet's disease. They present as erythema nodosum-like lesion, papulopustular eruption, erythema multiforme-like lesion, thrombophlebitis, skin ulcer, Sweet's syndrome-like lesion, bullous necrotizing vasculitis, and pyoderma gangrenosum. The hyperreactivity of the skin to any intracutaneous injection or needle prick, which is known as pathergy, is one clinically-unique feature of the disease.
Behcet's Syndrome/pathology* ; Human ; Skin/pathology*

Behcet's disease is recognized as a systemic inflammatory disease of unknown etiology. The disease has a chronic course with periodic exacerbations and progressive deterioration. Previous reports have shown at least three major pathophysiologic changes in Behcet's disease; excessive functions of neutrophils, vasculitis with endothelial injuries, and autoimmune responses. Many reports suggested that immunological abnormalities and neutrophil hyperfunction may be involved in the etiology and the pathophysiology of this disease. HLA-B51 molecules by themselves may be responsible, in part, for neutrophil hyperfunction in Behcet's disease. T cells in this disease proliferated vigorously in response to a specific peptide of human heat shock protein (hsp) 60 in an antigen-specific fashion. T cells reactive with self-peptides produced Th1-like proinflammatory and/or inflammatory cytokines. This leads to tissue injury, possibly via delayed-type hypersensitivity reaction, macrophage activation, and activation and/or recruitment of neutrophils. These data shed new light on the autoimmune nature of Behcet's disease; molecular mimicry mechanisms may induce and/or exacerbate Behcet's disease by bacterial antigens that have activated T cells which are reactive with self-peptide(s) of hsp. This would lead to positive selection of autoreactive T cells in this disease.
Behcet's Syndrome/pathology ; Behcet's Syndrome/immunology* ; Behcet's Syndrome/etiology ; Chaperonin 60/immunology ; Eye/pathology ; Human ; Neutrophils/physiology ; Skin/pathology ; T-Lymphocytes/physiology

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

PURPOSE: To evaluate contrast sensitivity in patients with Behçet's disease (BD) without ocular involvement. METHODS: The study group was composed of 47 BD patients (20 to 50 years of age) who did not have ocular involvement. The control group was composed of 47 normal volunteers who were similar to the study group in terms of age and gender. No participants in this study had any ocular or systemic pathologies except for BD. The contrast sensitivity measurements were performed using the Functional Acuity Contrast Test under photopic conditions, and the results were compared between the two groups. RESULTS: The mean age of the BD patients and control subjects was 34.5 ± 9.7 and 33.2 ± 7.6 years, respectively. The mean disease duration of the BD patients was 5.5 ± 6.4 years. There was a statistically significant decrease at five spatial frequencies (A, 1.5; B, 3; C, 6; D, 2; and E, 18 cycles per degree) in patients with BD compared with control subjects (p < 0.001, p = 0.004, p = 0.002, p < 0.001, and p = 0.001, respectively). CONCLUSIONS: The contrast sensitivity of BD patients without ocular involvement was lower than that of the control group. Further studies seem mandatory to confirm our results.
Behcet Syndrome ; Contrast Sensitivity ; Healthy Volunteers ; Humans ; Pathology

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

Behcet's disease (BD) is a chronic, relapsing, multisystem disorder, characterized by recurrent oral ulcer, genital ulcers, eye lesion, and skin lesion. The underlying pathology is nonspecific vasculitis of all vessel sizes, and severe vasculitis can result in fistula formation of neighboring tissues due to a necrotic process. Herein, eleven cases of BD combined with fistula are presented. In the present study, various types of fistula were associated; enterocutaneous fistula in six patients, and rectovaginal fistula in two. The other three patients showed aortoduodenal fistula, urethrovaginal fistula and urethrocutaneous fistula. They were treated with a corrective operation, but the prognoses were poor due to frequent relapses.
Humans ; Fistula/*etiology/surgery ; Female ; Behcet Syndrome/*complications/pathology ; Adult ; Adolescent

Adult ; Behcet Syndrome ; complications ; Female ; Heart Atria ; pathology ; Humans ; Myocardial Infarction ; complications ; Pericardial Effusion ; complications

No abstract available.
Behcet Syndrome/*pathology ; Female ; Humans ; Interleukins/*blood ; Male ; Receptors, Cell Surface/*blood

This study was done to evaluate the frequency, intensity, and specificity of a positive pathergy reaction (PR) in Behcet's disease (BD) patients, to clarify an association between the PR and the clinical features or disease severity, and to assess whether patients with pustule formation at the venous puncture site (PFVPS) without positive PR could be regarded as a positive reaction. The PR was tested in 64 BD patients, 74 disease controls, and 20 healthy controls. Venous PR was performed in 8 BD patients with PFVPS. Follow-up PR was done in 14 patients with positive reaction during inactive phase. The PR was positive in 35.9% of BD patients, in 1 patient among disease controls, and in none of healthy controls. The pustule formation was observed in one BD patient. There was no statistical significance between positive PR and the clinical variables. The mean clinical activity score of BD patients with positive PR was similar to patients with negative reaction. Venous PR was positive in 7 patients. The follow-up PR was positive in 2 patients during inactive phase. Conclusively the positive PR appeared to be specific for BD, and was not associated with the clinical variables or disease severity, but was usually found during active phase in cases with positive reaction. The PFVPS in patients with negative PR might be considered to be positive.
Adult ; Behcet Syndrome/*pathology ; Female ; Humans ; Male ; Middle Aged ; Sensitivity and Specificity ; Severity of Illness Index ; Skin/*pathology ; Suppuration