Pathergy Phenomenon

Frontiers in Medicine ◽

10.3389/fmed.2021.639404 ◽

2021 ◽

Vol 8 ◽

Author(s):

Tulin Ergun

Keyword(s):

Behçet’S Disease ◽

Complex Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Middle Eastern ◽

Adaptive Immune System ◽

Related Factors ◽

Behcet's Disease ◽

Sterile Needle ◽

Needle Prick

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.

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A Darwinian view of Behçet's disease

Rheumatology and Immunology Research ◽

10.2478/rir-2021-0013 ◽

2021 ◽

Vol 2 (2) ◽

pp. 91-99

Author(s):

Rhodri Smith ◽

Robert J. Moots ◽

Mariam Murad ◽

Graham R. Wallace

Keyword(s):

Behçet’S Disease ◽

Complex Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Clinical Findings ◽

Unknown Etiology ◽

Inflammatory Disorder ◽

Protective Mechanisms ◽

Behcet's Disease ◽

Cns Lesions

Abstract Behçet’s disease (BD) is a multisystem inflammatory disorder of unknown etiology, characterized by oral and genital ulceration, with other complications including eye, skin, joint, and central nervous system (CNS) lesions. Diagnosis is based on clinical findings, which may differ between patients. There is a strong genetic basis for BD; however, only a few genes have been associated with the disease across the geographical spread of BD. In this article, we discuss the history and combination of genes involved in this complex disease in relation to the geographical range and present our view that the disease has developed from a Darwinian perspective, with different gene polymorphisms that affect the same biological pathway. Moreover, these mutations individually are protective mechanisms against the disease relevant to each region, which affected both archaic and modern humans.

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Sexual dysfunction and depression in Behçet’s disease in comparison to healthy controls

Rheumatology International ◽

10.1007/s00296-021-05000-4 ◽

2021 ◽

Author(s):

Sebastian-Jonas Saur ◽

Alexandra Schlögl ◽

Torsten Schmalen ◽

Simon Krittian ◽

Ann-Christin Pecher ◽

...

Keyword(s):

Sexual Dysfunction ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Middle Eastern ◽

Control Group ◽

Healthy Controls ◽

Behcet's Disease ◽

Caucasian Origin ◽

Male Patients

AbstractBehçet’s disease (BD) can affect the genital system and is more common in Middle Eastern countries and Asia but also occurs in Caucasian people. Aim of this study was to evaluate the prevalence of sexual dysfunction (SD) and depression in patients with BD compared to a healthy control group (HCG). In addition, differences with regard to depression and patients’ origin were evaluated. This prospective, monocentric study included 106 consecutive patients from our specialized BD outpatient clinic. Patients were asked to fill out the paper based standardized and validated questionnaires International Index of Erectile Function (IIEF), the Female Sexual Function Index (FSFI) and the Beck Depression Inventory (BDI). In addition, 206 healthy controls were asked to fill out the questionnaires. 106 patients with BD were evaluated and 206 participants in the HCG. The mean age in BD group was 40.5 years as compared to 44.4 years in the HCG. Half of the patients had Middle Eastern and half Caucasian origin. SD was found in 24.5% of all subjects. Only 6.9% of male patients showed signs of SD, while half of the women’s group was suffering from SD. The prevalence for SD was significantly higher in women with Middle Eastern ethnic origin compared to women with Caucasian origin (75 vs. 33.3%, p = 0.024). Erectile Dysfunction occurred in 55% of all male patients which was not statistical different from the HCG. Genital ulcers affected 73.6% of all patients. Depression was found in 36.7% of all subjects as compared to 6.25% in the HCG (p < 0.001). Both, SD and depression correlated positively in males (p = 0.017) and females (p = 0.013). SD and depression are very common problems in BD and should be addressed by the treating physician. Both manifestations are intensifying each other. Depression especially is more prevalent compared to the healthy population.

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Behcet’s Disease: Is There Geographical Variation? A Review Far from the Silk Road

International Journal of Rheumatology ◽

10.1155/2015/945262 ◽

2015 ◽

Vol 2015 ◽

pp. 1-7 ◽

Cited By ~ 20

Author(s):

Nieves Marie Leonardo ◽

Julian McNeil

Keyword(s):

Behçet’S Disease ◽

Geographical Variation ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Wide Spectrum ◽

Systemic Vasculitis ◽

Middle Eastern ◽

Clinical Manifestations ◽

Behcet's Disease ◽

Cardiac Manifestations

Behcet’s Disease (BD) is a systemic vasculitis characterized by the triad of recurrent mouth and genital ulcers with eye involvement. To date there are no laboratory tests specific for the disease and diagnosis continues to remain on clinical grounds. Multiple criteria have been created as guides for diagnosis; however, given the wide spectrum of organ involvement, some cases remain undiagnosed. The diagnosis of Behcet’s Disease may only be made over time as the clinical manifestations emerge sometimes separated by months and even years. With an increased recognition of this disease it has become apparent that there is geographical variation in clinical manifestations. In particular cardiac manifestations are not seen commonly in Caucasians compared to Asian and Middle Eastern patients, while neurological manifestations are more common in Caucasians. Use of immunosuppressive and immunomodulatory drugs to suppress inflammation remains the cornerstone of treatment.

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HLA antigens associated with Behcet's disease

Archives of Dermatology ◽

10.1001/archderm.113.12.1720 ◽

1977 ◽

Vol 113 (12) ◽

pp. 1720-1721 ◽

Cited By ~ 4

Author(s):

F. Ersoy

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Hla Antigens ◽

Behcet's Disease

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Coagulation and Fibrinolytic Activity in Behçet's Disease

Thrombosis and Haemostasis ◽

10.1055/s-0038-1646409 ◽

1991 ◽

Vol 66 (03) ◽

pp. 292-294 ◽

Cited By ~ 57

Author(s):

K K Hampton ◽

M A Chamberlain ◽

D K Menon ◽

J A Davies

Keyword(s):

Plasminogen Activator ◽

Tissue Plasminogen Activator ◽

Behçet’S Disease ◽

Fibrinolytic Activity ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Plasminogen Activator Inhibitor ◽

Behcet's Disease ◽

Tissue Plasminogen ◽

Von Willebrand

SummaryCoagulation and fibrinolytic activities were studied in 18 subjects with Behçet's disease and compared with results from 14 matched control patients suffering from sero-negative arthritis. Significantly higher plasma concentrations (median and range) were found in Behçet's patients for the following variables: fibrinogen 3.7 (1.7-6.9) vs 3.0 (2.0-5.1) g/1, p <0.05; von Willebrand factor antigen, 115 (72-344) vs 74 (60-119)%, p <0.002; plasminogen activator activity (106/ECLT2) 219 (94-329) vs 137 (78-197) units, p <0.002; tissue plasminogen activator inhibitor (t-PA-I) activity, 9.1 (5.5-19.3) vs 5.1 (1.8-12.0) IU/ml, p <0.002; and PAI-1 antigen, 13.9 (4.5-20.9) vs 6.4 (2.4-11.1) ng/ml, p <0.002. Protein C antigen was significantly lower: 97 (70-183) vs 126 (96-220)%, p <0.02. No differences were observed in antithrombin III activity or antigen, factor VIII coagulant activity, fibrinopeptides A and Bβ15-42, plasminogen, α-2-antiplasmin, functional and immunological tissue-plasminogen activator, thrombin-antithrombin complexes and D-dimer. Levels of tissue plasminogen activator inhibitor (activity and antigen) correlated with disease activity while fibrinogen and von Willebrand factor concentrations did not. Seven of the 18 subjects with Behçet's disease had suffered thrombotic events but it was not possible to distinguish these from the 11 patients without thrombosis using the assays performed. The results suggest the abnormal fibrinolytic activity in Behçet's disease is due to increased inhibition of tissue plasminogen activator. No abnormality of coagulation or fibrinolytic activity specific to Behçet's disease was detected.

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