Only genital aphthae and erythema nodosum were more frequent in females. On the other hand papulopustular eruptions, thrombophlebitis, ocular, neurologic, pulmonary and vascular involvement were more frequent in males. While female patients had the best prognosis, male patients had a worse overall prognosis than females.

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Behçet's disease is a multisystemic disorder characterized by recurrent ulcers of the mouth and genitalia and relapsing iritis. Four types of vascular lesion are recognized in Behçet's disease: arterial occlusions, aneurysms, venous occlusions, and variceal development. The incidence of vascular involvement reported in the literature ranges from 7% to 29%. The aim of this study was to determine the rate of vascular involvement in Behçet's disease at our hospital between 1983 and 1992. Of 1200 patients with Behçet's disease, 173 (14.4%) had venous manifestations and 19 (1.6%) had arterial manifestations (in some patients more than one organ was involved). In the group of patients with venous manifestations, there were 154 (12.8%) with venous thrombosis, 17 (1.4%) with vena cava superior syndrome, 5 (0.4%) with inferior vena cava syndrome, 5 (0.4%) with varices, 2 with upper extremity venous thrombosis, 1 with internal jugular vein thrombosis, 1 with cavernous sinus thrombosis, and 1 with hepatic vein thrombosis. In the arterial manifestation group there were 7 femoral, 3 abdominal, 3 popliteal, 2 iliac, 2 pulmonary, 1 axillary, and 1 carotid artery aneurysm as well as 3 arterial occlusions. We concluded that vascular surgeons dealing with young adults should bear Behçet's disease, an uncommon clinical entity, in mind.

To evaluate the prevalence of the clinical findings in Behçet's disease, we retrospectively analyzed the clinical data of 2147 Behçet patients from 9 to 87 years of age (mean age 38.3 years) followed up during the years 1976 through 1997. One thousand ninety three patients were male and 1054 patients were female. The male/female ratio was 1.03. The mean age at onset was 25.6 years. A family history of Behçet's disease was present in 7.3% of the patients. Positive pathergy was found in 1220 (56.8%) patients. All of the patients had mucocutaneous lesions. Out of the 2147 patients the disease manifested itself as only mucocutaneous involvement in 1168 patients. The prevalence of systemic manifestations was found as follows: 28.9% ocular involvement, 16.0% musculoskeletal involvement, 16.8% vascular involvement, 2.8% gastrointestinal involvement, 2.2% neurological involvement. Pulmonary involvement was seen in 20 (1.0%) patients, cardiac involvement was seen in 3 patients and renal involvement was observed in 2 patients. Male patients had vascular involvement 5.02, neurologic involvement 2.21 and ocular involvement 1.98 times more frequently than female patients.

Behçet disease should be considered in the differential diagnosis of childhood uveitis, particularly in endemic areas. The collaboration of pediatricians, rheumatologists, dermatologists, ophthalmologists, and other specialists when necessary is required in the diagnosis and management of children with Behçet disease.

The existing regional prevalance studies conducted in Turkey have indicated that the real number of Behçet's patients in our country is markedly higher than the number of registered patients. Therefore the National Behçet's Disease Commity and Surveillance System was founded by our research group in December 1999.

Summary Background Behçet disease (BD) is an immune‐mediated vasculitis‐like syndrome characterized by recurrent aphthous lesions and various systemic manifestations. Inflammatory markers may be useful to assess disease severity. The Systemic Immune‐Inflammation Index (SII) (neutrophils × platelets/lymphocytes) has been widely used in oncology since 2014, with promising results. Aim To assess the efficiency of the SII in determining activity of BD. Methods This retrospective cohort study was conducted on patients with BD who were admitted to the outpatient clinic of the Department of Dermatology and Venereology, Ufuk University Hospital, between 1 January 2010 and 31 December 2019. Patients were divided into two groups based on their disease status upon admission: (i) active BD (n = 103), and (ii) inactive BD (n = 63). Clinical characteristics, demographic features, type of medications, full blood count parameters, erythrocyte sedimentation rate (ESR), C‐reactive protein (CRP), ferritin and SII were compared between the groups. Furthermore, receiver operating characteristic curve analysis was performed to assess the performance of the SII in determining disease severity upon admission to hospital. Results Higher numbers of white blood cells, platelets and neutrophils, greater red cell distribution width, higher levels of ESR, CRP and ferritin, and higher SII were observed in the active disease group (P < 0.001). The cutoff value of 552 × 103/mm3 was found to have 81% sensitivity and 82% specificity. Conclusion The SII may be used as an additional indicator for the assessment of BD status and physicians should be cautious in patients with SII levels of > 552 × 103/mm3) at the initial evaluation of the patients.

Sixty-three patients with Behçet's disease (BD), 30 patients with recurrent oral ulcer and 30 healthy individuals as control group were included in the study. ISG criteria was used for the diagnosis of BD and patients were classified as active and inactive and evaluated accordingly. In the patient and control groups, von Willebrand factor (vWF), tissue plasminogen activator (tPA), and plasminogen activator inhibitor (PAI) levels were determined using ELISA method and antithrombin III (AT-III) by nephelometric methods. High levels of endothelial product, vWF in the active Behçet patient group (p < 0.005) supports endothelial destruction due to vasculitis related with BD. In the active patient group tPA levels were significantly lower (p < 0.05) than the inactive and control groups with higher levels of PAI (p < 0.05 and p < 0.01) respectively. In Behçet disease, besides the decrease in tPA synthesis, high PAI levels also can affect tPA decrease and lead to inhibition of fibrinolytic activity. In active Behçet group, levels of AT-III were low and no significant difference was observed in recurrent oral ulcer and control groups. This situation may arise from the excessive use of AT-III in active disease. In conclusion, high levels of vWF in Behçet patients is thought to arise from vasculitis and high levels of PAI from the accumulation of thrombocytes on the damaged surface of endothelium leading to a decrease in tPA levels and inhibition of fibrinolytic activity.