Mucocutaneous criteria for the diagnosis of Behçet's disease: An analysis of clinicopathologic data from multiple international centers

Jorizzo, Joseph L.; Abernethy, John Leo; White, Wain L.; Mangelsdorf, Heidi C.; Zouboulis, Christos C.; Sarıca, Rıfkıye; Gaffney, Karl; Mat, Cem; Yazıcı, Hasan; Ialaan, Abdullah Al; Assad-Khalil, Samir Helmy; Kaneko, Fumio; Jorizzo, Ernest A Frederick

doi:10.1016/0190-9622(95)91333-5

Cited by 115 publications

(74 citation statements)

References 9 publications

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“…It has been suggested that immunoactive cells such as T cells infiltrate into the affected regions with activation of circulating T and B lymphocytes, followed by chemotaxis of neutrophils or vice versa [16, 17, 18]. The histologic features are characterized by inflammatory processes in the arteries and veins and thrombosis as a result of vasculitis of the vasa vasorum with thickening of the media [16, 17, 19, 20]. The intermittent nature of the disease and the lack of consistent response to therapy make the underlying etiology difficult to define.…”

Section: Discussionmentioning

confidence: 99%

Increased Plasma Adrenomedullin Levels in Patients with Behçet’s Disease

Evereklioğlu

Yürekli²,

et al. 2000

Dermatology

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Background: Behçet’s disease (BD) is a chronic systemic inflammatory disorder affecting multiple organs with a generalized vasculitis of arteries and veins. Endothelial dysfunction is one of the prominent features of BD. Adrenomedullin (AM) is a peptide produced not only in normal adrenal medulla but also in the vascular smooth muscle cells and endothelial cells, and its role in the course of BD has not been previously described. Objective: To detect changes of plasma AM concentrations in patients with BD compared with age- and sex-matched healthy subjects by using high-performance liquid chromatography (HPCL). We also investigated if disease activity or the duration of BD correlates with AM levels. Methods: Forty-two consecutive patients with BD (38.5 ± 11.1 years, 19 male and 23 female) and 20 healthy age- and sex-matched control subjects (39.5 ± 10.9 years, 8 male and 12 female) were included in this study. We measured plasma AM levels by HPCL, and acute-phase reactants including α₁-antitrypsin and α₂-macroglobulin, neutrophil count and the erythrocyte sedimentation rate. Results: Mean ± SD plasma AM levels in patients with BD (73.22 ± 25.55 pmol/l) were significantly higher (p < 0.001) than in healthy control volunteers (21.35 ± 12.37 pmol/l). Patients with active BD had similar plasma AM concentrations (79.32 ± 21.89 pmol/l) with patients with inactive disease (67.44 ± 29.92 pmol/l). On the other hand, patients with longer duration of the disease (mean duration, 13.9 ± 3.8 years) had significantly higher plasma AM levels (83.99 ± 19.71 pmol/l; p = 0.005) than patients (62.45 ± 26.57 pmol/l) with shorter duration of the disease (mean duration, 5.5 ± 2.3 years). All acute-phase reaction parameters were found to be significantly increased in the active disease. Conclusion: Considering its endothelial cell implications, AM may be involved in reparatory vessel endothelium mechanisms, especially in the chronic disease.

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Section: Discussionmentioning

confidence: 99%

Increased Plasma Adrenomedullin Levels in Patients with Behçet’s Disease

Evereklioğlu

Yürekli²,

et al. 2000

Dermatology

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“…Pathergy reaction, which is a non-specific cutaneous hypersensitive response showing around 2 mm pustule 24-48 h after pricking with 20 G syringe needle, has been thought to be helpful to diagnose BD, because the phenomenon has been believed as a unique feature of BD described by International Study Group of BD [40] . Histology and immunohistology of the "Pathergy reaction" is similar to those of EN-like eruption from BD patients [41,42] . However, recently the reactivity by Pathergy test became chronologically lower to less than 40% of BD patients seen in 2007s, though the response was seen in more than 70% of the patients in 1970s.…”

Section: Pathergy Test and Oral Streptococcimentioning

confidence: 73%

“…BD symptoms are characterized by vascular involvements histologically showing swollen endothelial cells of the micro-veins infiltrated by inflammatory monocytes with a few neutrophils, so-called "vascular reaction" seen in EN-like eruption and other lesions [17,42,78,79] . The strong hypersensitivity reaction against S. sanguinis agents carried by APCs can be suspected in the pathogenesis of BD which may be one of the extrinsic triggering factors [11,12,17,18,20] .…”

Section: Ras and Systemic Symptomsmentioning

confidence: 99%

Behcet’s Disease and Related Diseases -Immune Reactions to Oralstreptococci in Their Pathogenesis

Kaneko

Togashi

Nomura

et al. 2016

Journal of Dermatological Research

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the retinal protein Brn3b which might be connected with the eye involvement in BD patients. Also, the peptides seem to be homologous with HSP-65 in association with the human HSP-60 which reactively appeared in serum of the patients involved by S. sanguinis.Then, the pathogenesis of BD was conclusively discussed on the relationship between RAS and the systemic symptoms by the vascular reaction due to immune responses against antigens derived from S. sanguinis. Non-BD patients with RAS and/or Lipschutz genital ulceration were weekly sensitized by oral streptococci, except for patients with EN. [2][3][4] .Although the actual etiology of BD is still unclear, the pathogenesis has been generally clearer by the etiological studies based on the genetic intrinsic factors and extrinsic triggering factors [5][6][7][8][9][10][11][12][13][14][15][16] . As one of the triggering factors, the oral unhygienic condition may be suspected, because periodontitis, decayed teeth, chronic tonsillitis, etc. are frequently noted in BD patients [12,13] . The proportion of Streptococcus sanguinis (S. sanguinis), which was previously recognized as species of the genus Streptococcus named "S. sanguis", was significantly high ABSTRACTBehçet's disease (BD) is a systemic disorder characterized by the recurrent involvement in the muco-cutaneous, ocular, intestinal, vascular, and/or nervous system organs. The clinical muco-cutaneous manifestations including recurrent aphthous stomatitis (RAS), erythema nodosum (EN) -like eruption, genital ulceration, etc. of patients with BD were reviewed in their pathogenesis comparing with the similar symptoms seen in patients without BD (non-BD). Most of BD patients tend to have hypersensitivity against streptococci which might be acquired in the oral cavity through the innate immune mechanism. Generally, BD patients have the systemic symptoms following RAS symptom as an immune reaction. Then, the characteristics of hypersensitivity to oral streptococci may be utilized in order to make a diagnosis for BD. The skin prick with self-saliva including oral streptococci was much more sensitive than "Pathergy test" conventionally used for BD diagnosis. HLA-B51-restricted CD8+ T cell response is suspected to catch the target tissues expressing major histocompatibility complex class 1 chain-related gene A (MICA) by stress in active BD patients. Bes-1 gene and 65kD of heat shock protein (HSP-65) derived from Streptococcus sanguinis (S. sanguinis) are detectable in the lesions. The peptides of Bes-1 gene are highly homologous with REVIEW

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“…[6][7][8] According to the international criteria, the diagnosis of Behçet's disease requires the presence of recurrent oral ulceration in the absence of other clinical explanations, and two of the following; recurrent genital ulceration, eye lesions, skin lesions and/or a pathergy test. [6][7][8] Although Behçet's disease has worldwide distribution, it is rare in the Americas and Europe and is more prevalent in Turkey, the Middle East and the Far East. It mainly affects young adults, and men have more severe vascular complications with this disease.…”

Section: Behçet's Diseasementioning

confidence: 99%

Budd-Chiari syndrome in association with Behçet's disease: review of the literature

et al. 2011

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The risk that patients with Behçet's disease may develop various thrombotic complications has been previously described. Although vascular complications from Budd-Chiari syndrome associated with Behçet's disease have been described, the pathogenic mechanisms are still unknown. Severe vascular complications present in Budd-Chiari syndrome associated with Behçet's disease are very common among young male adults. The objective of this study was to review the literature and present the association of Budd-Chiari syndrome with Behçet's disease.

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Mucocutaneous criteria for the diagnosis of Behçet's disease: An analysis of clinicopathologic data from multiple international centers

Cited by 115 publications

References 9 publications

Increased Plasma Adrenomedullin Levels in Patients with Behçet’s Disease

Increased Plasma Adrenomedullin Levels in Patients with Behçet’s Disease

Behcet’s Disease and Related Diseases -Immune Reactions to Oralstreptococci in Their Pathogenesis

Budd-Chiari syndrome in association with Behçet's disease: review of the literature

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