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      Call for Papers: Digital Platforms and Artificial Intelligence in Dementia

      Submit here by August 31, 2025

      About Dementia and Geriatric Cognitive Disorders: 1.9 Impact Factor I 5.3 CiteScore I 0.781 Scimago Journal & Country Rank (SJR)

      Call for Papers: Skin Health in Aging Populations

      Submit here by December 31, 2025

      About Skin Pharmacology and Physiology: 3.2 Impact Factor I 6.6 CiteScore I 0.833 Scimago Journal & Country Rank (SJR)

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      A Comparison of Adult and Juvenile Behcet Patients and a Look at Clinical Trends: Retrospective Data from a Turkish Follow-Up Cohort Study

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          Abstract

          Background: Previous reports indicate that juvenile Behçet’s disease (BD) may have a different course than adult BD. However, as a direct comparison with adult Behçet patients has only been made in a limited number of studies, the issue is still controversial. Objectives: The primary aim of our study was to compare clinical manifestations in a large cohort of juvenile and adult Behçet patients registered in a single centre. The secondary aim of our study was to compare the data of newly diagnosed patients registered between 1998 and 2020 with the data of those registered between 1976 and 1997. Methods: Data were collected retrospectively from medical records of patients registered between 1998 and 2020. Juvenile BD was defined as fulfilment of International Criteria for Behçet’s Disease at or before 16 years of age. Results: A similar course of disease was noted in juvenile and adult Behçet patients with no significant difference in the frequency of mucocutaneous findings, major organ involvement, and positivity of the pathergy test. A comparison of the periods, 1976–1997 and 1998–2020, revealed no significant difference in the prevalence of mucocutaneous lesions and major organ involvement. Conclusions: Our results indicate that juvenile and adult Behçet patients have a similar course with a similar frequency of clinical manifestations. Contrary to reports suggesting an overall tendency to milder disease over time, no decrease in the risk of major organ involvements was observed. A significant trend towards a decline in pathergy test positivity was noted.

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          Most cited references48

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          Demographic and Clinical Characteristics of Uveitis in Turkey: The First National Registry Report.

          To describe the demographic and clinical profiles of uveitis patients seen at secondary and tertiary care centers in Turkey.
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            Twenty-Eight Cases of Juvenile-Onset Adamantiades-Behçet Disease in Germany

            Background and Objective: Adamantiades-Behçet disease is a rare entity at a juvenile age. We aimed to enlighten epidemiological and clinical characteristics of juvenile-onset disease in Germany. Methods: Data from the German Registry were used to compare clinical and epidemiological features of patients with juvenile-onset (≤16 years) and adult-onset (>16 years) disease diagnosed according to the criteria of the International Study Group. Results: Twenty-eight (17%) of 168 patients of the German Registry exhibited the onset of the disease and 8 (5%) of them the complete symptom complex at a juvenile age. Juvenile-onset disease was characterized by an increase in familial cases (25 vs. 8% in patients with adult-onset; p = 0.047). The frequency of diagnostic signs was similar between the two study groups. In juvenile-onset disease, delayed development of the complete symptom complex (median value 35 months vs. 12 months after onset; p = 0.014) and lower prevalence of severe complications (9 vs. 29%; p = 0.042) were detected. Conclusions: The major clinical features of juvenile-onset and adult-onset disease in Germany are comparable, but in juvenile-onset disease, the course is delayed and patients experience less severe complications. In addition, there is a higher rate of familial occurrence of the disease in patients with juvenile-onset.
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              Is Open Access

              Pathergy Phenomenon

              Skin pathergy reaction (SPR) is a hyperreactivity response to needle induced trauma which is characterized by a papule or pustule formation, 24–48 h after sterile-needle prick. It is affected by a wide array of factors, including the physical properties of the needles being used, number of pricks and disease related factors such as male gender, active disease. There is a great variation in reactivity among different populations with very low positivity rate in regions of low prevalence like Northern Europe, and higher prevalance mainly in communities living along the historical Silk Road, like Turkey, China and Middle Eastern countries. SPR is not constant during the disease course, has lost its sensitivity over decades and can be positive in various disorders including Sweet's syndrome, pyoderma gangrenosum, Crohn's diesease, A20 haploinsufficiency, deficiency of IL-1-receptor antagonist and few others. Nevertheless, it is a criteria included into many currently used diagnostic or classification criteria for Behçet's disease. Although, not being fully uncovered yet, available data points to the activation of both innate and adaptive immune system with an inflammatory response mediated by polymorphonuclears and T-cells. In addition to its utility in diagnosis of Behçet's Disease, SPR may serve as a model for investigating the inflammatory pathways involved in the etiopathogenesis of this complex disease.

                Author and article information

                Journal
                DRM
                Dermatology
                10.1159/issn.1018-8665
                Dermatology
                Dermatology
                S. Karger AG
                1018-8665
                1421-9832
                2023
                December 2023
                04 October 2023
                : 239
                : 6
                : 958-965
                Affiliations
                [a ]Department of Dermatology, Faculty of Medicine, Ankara University, Ankara, Turkey
                [b ]Department of Internal Medicine, Division of Rheumatology, Faculty of Medicine, Ankara University, Ankara, Turkey
                [c ]Department of Ophthalmology, Faculty of Medicine, Ankara University, Ankara, Turkey
                [d ]Department of Surgery, Baylor College of Medicine/The Methodist Hospital, Houston, Texas, USA
                [e ]Department of Neurology, Faculty of Medicine, Ankara University, Ankara, Turkey
                Author notes
                *Ayse Oktem, ayseoktem@yahoo.com
                Article
                534316 Dermatology 2023;239:958–965
                10.1159/000534316
                37793347
                88f10b40-26a2-4074-9588-728239355397
                © 2023 S. Karger AG, Basel
                History
                : 23 November 2022
                : 24 September 2023
                Page count
                Tables: 4, Pages: 8
                Funding
                The authors received no financial support for the research, authorship, and/or publication of this article.
                Categories
                Research Article

                Medicine
                Juvenile Behçet’s disease,Clinical manifestations,Behçet’s disease
                Medicine
                Juvenile Behçet’s disease, Clinical manifestations, Behçet’s disease

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