Behcet Syndrome ; complications ; Hemorrhage ; etiology ; Humans ; Pharynx

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

Behcet's disease is recognized as a multisystemic disease with various organ involvement including skin, mucous membrane, eyes, joints, veins, arteries, gastrointestinal tract, meninges, and brain. In this review, Behcet's disease has been approached from two rheumatologic perspectives--as an intermittent and periodic arthritic syndrome and as a vasculitic syndrome. Association with seronegative spondyloarthropathy and other autoimmune diseases will also be discussed.
Arthritis/etiology* ; Autoimmune Diseases/etiology* ; Behcet's Syndrome/complications* ; Human ; Vasculitis/etiology*

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

Adolescent ; Arterial Occlusive Diseases ; complications ; Behcet Syndrome ; complications ; Humans ; Hypertension ; etiology ; Hypertensive Encephalopathy ; etiology ; Male

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 ; diagnosis ; Blood Sedimentation ; C-Reactive Protein ; analysis ; Cardiovascular Diseases ; etiology ; Humans ; Male

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

Adult ; Behcet Syndrome ; complications ; Cochlear Implantation ; Cochlear Implants ; Deafness ; etiology ; rehabilitation ; Humans ; Male ; Treatment Outcome

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