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Fereydoun Davatchi - One of the best experts on this subject based on the ideXlab platform.

  • Dermatoscopic and Mucoscopic Features of Lesions in Patients with Behcet's Disease.
    Acta reumatologica portuguesa, 2019
    Co-Authors: Seyedeh Zahra Ghodsi, Farhad Shahram, Fereydoun Davatchi, Abdolhadi Nadji, H. Chams, Bahrololoumi N. Bafruee, Chams C. Davatchi, Cliff Rosendahl, Bengü Nisa Akay, Maassoomeh Akhlaghi
    Abstract:

    Objective: Behcet's disease (BD), as a vasculitis, can affect small and large vessels. As dermatoscopy has been shown to improve the accuracy in diagnosis of various skin lesions especially vascular patterns, we set this study to find if there is any characteristic pattern in the dermatoscopy of Behcet's mucocutaneous lesions.Methods: This prospective cross-sectional observational study designed to evaluate dermatoscopic features of Behcet's mucocutaneous lesions. Fifty six consecutive patients presenting at the outpatient clinic of the BD Research Unit were included. If present, for each patient one oral, one skin and one genital lesion were evaluated by dermatoscopy. When indicated, Pathergy test was performed according to a standard protocol and the results were evaluated by dermatoscopy.Results: A total of 40 oral, 8 genital, 14 skin lesions and 14 Pathergy tests were evaluated by dermatoscopy. While vascular component was the most prominent feature in oral aphthae, this component was less prominent in genital lesions. Dot vessels were the most common form of vessels in both oral and skin lesions. All the oral lesions were characterized by a central white structureless area. Skin lesions were characterized by a red structureless background. In Pathergy tests, negative pricks showed absence of specific features while positive pricks were characterized by a structureless background in pink, purple or red. No obvious vascular component was detected in any of the pricks.Conclusion: It seems that these findings have no specific clues for the diagnosis of BD, but our study is the first study in this field and the findings may give way to further investigations.

  • The association of Pathergy reaction and active clinical presentations of Behçet's disease.
    Reumatologia, 2017
    Co-Authors: Shirin Assar, Farhad Shahram, Bahar Sadeghi, Fereydoun Davatchi, Seyyedeh Zahra Ghodsi, Abdolhadi Nadji, Farimah Ashofte, Seyyedeh Roghieh Larimi, Masoud Sadeghi
    Abstract:

    OBJECTIVES The Pathergy skin test is a hypersensitivity reaction to a prick skin trauma caused by a pin or a needle, which is considered as a specific presentation in Behcet's disease (BD) and the precise mechanism of this test is not well elucidated. This study was designed to evaluate the association of Pathergy reaction (PR) with the active clinical manifestations of BD patients, to assess the clinical importance of PR. MATERIALS AND METHODS This was a cohort study on 1675 BD patients who fulfilled the entry criteria based on the International Criteria for Behcet's Disease (ICBD) from 1975 to 2011. The patients were divided into two groups; the Pathergy positive group included 841 patients (50.2%) and the Pathergy negative group 834 patients (49.8%). The active mucocutaneous and systemic disease manifestations were analyzed according to the presence of the PR. The odds ratio and 95% confidence interval (95% CI) were calculated for each item. RESULTS In this study, 841 patients (50.2%) had a positive Pathergy test. Patient's mean age and mean disease duration were similar in the two groups (Pathergy positive and Pathergy negative patients). There was no association between positive Pathergy test and age of patients, or disease duration. The PR was associated with male gender (p = 0.013), oral aphthosis (p < 0.001), pseudofolliculitis (p < 0.001), anterior uveitis (p = 0.001) and posterior uveitis (p = 0.028). CONCLUSIONS The presence of PR was associated with male gender, as well as some of mucocutaneous manifestations and uveitis in adult patients. There was no association with retinitis and vascular involvements. PR isn't associated with the severity of the disease.

  • The association of Pathergy reaction and active clinical presentations of Behçet’s disease
    Reumatologia Rheumatology, 2017
    Co-Authors: Shirin Assar, Farhad Shahram, Bahar Sadeghi, Fereydoun Davatchi, Seyyedeh Zahra Ghodsi, Abdolhadi Nadji, Farimah Ashofte, Seyyedeh Roghieh Larimi, Masoud Sadeghi
    Abstract:

    Objectives : The Pathergy skin test is a hypersensitivity reaction to a prick skin trauma caused by a pin or a needle, which is considered as a specific presentation in Behçet’s disease (BD) and the precise mechanism of this test is not well elucidated. This study was designed to evaluate the association of Pathergy reaction (PR) with the active clinical manifestations of BD patients, to assess the clinical importance of PR. Materials and methods : This was a cohort study on 1675 BD patients who fulfilled the entry criteria based on the International Criteria for Behçet’s Disease (ICBD) from 1975 to 2011. The patients were divided into two groups; the Pathergy positive group included 841 patients (50.2%) and the Pathergy negative group 834 patients (49.8%). The active mucocutaneous and systemic disease manifestations were analyzed according to the presence of the PR. The odds ratio and 95% confidence interval (95% CI) were calculated for each item. Results : In this study, 841 patients (50.2%) had a positive Pathergy test. Patient’s mean age and mean disease duration were similar in the two groups (Pathergy positive and Pathergy negative patients). There was no association between positive Pathergy test and age of patients, or disease duration. The PR was associated with male gender (p = 0.013), oral aphthosis (p < 0.001), pseudofolliculitis (p < 0.001), anterior uveitis (p = 0.001) and posterior uveitis (p = 0.028). Conclusions : The presence of PR was associated with male gender, as well as some of mucocutaneous manifestations and uveitis in adult patients. There was no association with retinitis and vascular involvements. PR isn’t associated with the severity of the disease

  • the international criteria for behcet s disease icbd a collaborative study of 27 countries on the sensitivity and specificity of the new criteria
    Journal of The European Academy of Dermatology and Venereology, 2014
    Co-Authors: Fereydoun Davatchi, S Assaadkhalil, Kenneth T Calamia, J E Crook, B Sadeghiabdollahi, Michael Schirmer, T Tzellos, Christos C Zouboulis, M Akhlagi, A Aldalaan
    Abstract:

    Objective Behcet's disease (BD) is a chronic, relapsing, inflammatory vascular disease with no pathognomonic test. Low sensitivity of the currently applied International Study Group (ISG) clinical diagnostic criteria led to their reassessment. Methods An International Team for the Revision of the International Criteria for BD (from 27 countries) submitted data from 2556 clinically diagnosed BD patients and 1163 controls with BD-mimicking diseases or presenting at least one major BD sign. These were randomly divided into training and validation sets. Logistic regression, ‘leave-one-country-out’ cross-validation and clinical judgement were employed to develop new International Criteria for BD (ICBD) with the training data. Existing and new criteria were tested for their performance in the validation set. Results For the ICBD, ocular lesions, oral aphthosis and genital aphthosis are each assigned 2 points, while skin lesions, central nervous system involvement and vascular manifestations 1 point each. The Pathergy test, when used, was assigned 1 point. A patient scoring ≥4 points is classified as having BD. In the training set, 93.9% sensitivity and 92.1% specificity were assessed compared with 81.2% sensitivity and 95.9% specificity for the ISG criteria. In the validation set, ICBD demonstrated an unbiased estimate of sensitivity of 94.8% (95% CI: 93.4–95.9%), considerably higher than that of the ISG criteria (85.0%). Specificity (90.5%, 95% CI: 87.9–92.8%) was lower than that of the ISG-criteria (96.0%), yet still reasonably high. For countries with at least 90%-of-cases and controls having a Pathergy test, adding 1 point for Pathergy test increased the estimate of sensitivity from 95.5% to 98.5%, while barely reducing specificity from 92.1% to 91.6%. Conclusion The new proposed criteria derived from multinational data exhibits much improved sensitivity over the ISG criteria while maintaining reasonable specificity. It is proposed that the ICBD criteria to be adopted both as a guide for diagnosis and classification of BD.

  • Classification and Diagnosis Criteria for Behçet’s Disease
    Behçet's Syndrome, 2013
    Co-Authors: Fereydoun Davatchi, Farhad Shahram, Bahar Sadeghi Abdollahi, Cheyda Chams-davatchi, Hormoz Shams, Abdolhadi Nadji
    Abstract:

    Behcet’s disease is relatively a young disease, described in 1937. Nevertheless, it has already 17 sets of diagnosis/classification criteria. The first set was created in 1946 and the last in 2010. The first created by an international collaboration was the International Study Group criteria (ISG) in 1990 with the collaboration of 7 countries, and the last the International Criteria for Behcet’s Disease (ICBD) in 2010 with the collaboration of 27 countries. In ICBD, the following signs get each 2 points: oral aphthosis, genital aphthosis, and ocular manifestations. Skin manifestations, vascular lesions, neurologic manifestations, and a positive Pathergy test get each 1 point. To classify or diagnose a patient as Behcet’s disease, the patient must totalize 4 or more points. As Pathergy test is not performed in some countries, mainly western countries, its use is facultative. However, in patients not fulfilling the ICBD it is recommended to perform it. It is also recommended to classify or diagnose a patient if no other disease could explain the symptoms.

Farhad Shahram - One of the best experts on this subject based on the ideXlab platform.

  • Dermatoscopic and Mucoscopic Features of Lesions in Patients with Behcet's Disease.
    Acta reumatologica portuguesa, 2019
    Co-Authors: Seyedeh Zahra Ghodsi, Farhad Shahram, Fereydoun Davatchi, Abdolhadi Nadji, H. Chams, Bahrololoumi N. Bafruee, Chams C. Davatchi, Cliff Rosendahl, Bengü Nisa Akay, Maassoomeh Akhlaghi
    Abstract:

    Objective: Behcet's disease (BD), as a vasculitis, can affect small and large vessels. As dermatoscopy has been shown to improve the accuracy in diagnosis of various skin lesions especially vascular patterns, we set this study to find if there is any characteristic pattern in the dermatoscopy of Behcet's mucocutaneous lesions.Methods: This prospective cross-sectional observational study designed to evaluate dermatoscopic features of Behcet's mucocutaneous lesions. Fifty six consecutive patients presenting at the outpatient clinic of the BD Research Unit were included. If present, for each patient one oral, one skin and one genital lesion were evaluated by dermatoscopy. When indicated, Pathergy test was performed according to a standard protocol and the results were evaluated by dermatoscopy.Results: A total of 40 oral, 8 genital, 14 skin lesions and 14 Pathergy tests were evaluated by dermatoscopy. While vascular component was the most prominent feature in oral aphthae, this component was less prominent in genital lesions. Dot vessels were the most common form of vessels in both oral and skin lesions. All the oral lesions were characterized by a central white structureless area. Skin lesions were characterized by a red structureless background. In Pathergy tests, negative pricks showed absence of specific features while positive pricks were characterized by a structureless background in pink, purple or red. No obvious vascular component was detected in any of the pricks.Conclusion: It seems that these findings have no specific clues for the diagnosis of BD, but our study is the first study in this field and the findings may give way to further investigations.

  • The association of Pathergy reaction and active clinical presentations of Behçet's disease.
    Reumatologia, 2017
    Co-Authors: Shirin Assar, Farhad Shahram, Bahar Sadeghi, Fereydoun Davatchi, Seyyedeh Zahra Ghodsi, Abdolhadi Nadji, Farimah Ashofte, Seyyedeh Roghieh Larimi, Masoud Sadeghi
    Abstract:

    OBJECTIVES The Pathergy skin test is a hypersensitivity reaction to a prick skin trauma caused by a pin or a needle, which is considered as a specific presentation in Behcet's disease (BD) and the precise mechanism of this test is not well elucidated. This study was designed to evaluate the association of Pathergy reaction (PR) with the active clinical manifestations of BD patients, to assess the clinical importance of PR. MATERIALS AND METHODS This was a cohort study on 1675 BD patients who fulfilled the entry criteria based on the International Criteria for Behcet's Disease (ICBD) from 1975 to 2011. The patients were divided into two groups; the Pathergy positive group included 841 patients (50.2%) and the Pathergy negative group 834 patients (49.8%). The active mucocutaneous and systemic disease manifestations were analyzed according to the presence of the PR. The odds ratio and 95% confidence interval (95% CI) were calculated for each item. RESULTS In this study, 841 patients (50.2%) had a positive Pathergy test. Patient's mean age and mean disease duration were similar in the two groups (Pathergy positive and Pathergy negative patients). There was no association between positive Pathergy test and age of patients, or disease duration. The PR was associated with male gender (p = 0.013), oral aphthosis (p < 0.001), pseudofolliculitis (p < 0.001), anterior uveitis (p = 0.001) and posterior uveitis (p = 0.028). CONCLUSIONS The presence of PR was associated with male gender, as well as some of mucocutaneous manifestations and uveitis in adult patients. There was no association with retinitis and vascular involvements. PR isn't associated with the severity of the disease.

  • The association of Pathergy reaction and active clinical presentations of Behçet’s disease
    Reumatologia Rheumatology, 2017
    Co-Authors: Shirin Assar, Farhad Shahram, Bahar Sadeghi, Fereydoun Davatchi, Seyyedeh Zahra Ghodsi, Abdolhadi Nadji, Farimah Ashofte, Seyyedeh Roghieh Larimi, Masoud Sadeghi
    Abstract:

    Objectives : The Pathergy skin test is a hypersensitivity reaction to a prick skin trauma caused by a pin or a needle, which is considered as a specific presentation in Behçet’s disease (BD) and the precise mechanism of this test is not well elucidated. This study was designed to evaluate the association of Pathergy reaction (PR) with the active clinical manifestations of BD patients, to assess the clinical importance of PR. Materials and methods : This was a cohort study on 1675 BD patients who fulfilled the entry criteria based on the International Criteria for Behçet’s Disease (ICBD) from 1975 to 2011. The patients were divided into two groups; the Pathergy positive group included 841 patients (50.2%) and the Pathergy negative group 834 patients (49.8%). The active mucocutaneous and systemic disease manifestations were analyzed according to the presence of the PR. The odds ratio and 95% confidence interval (95% CI) were calculated for each item. Results : In this study, 841 patients (50.2%) had a positive Pathergy test. Patient’s mean age and mean disease duration were similar in the two groups (Pathergy positive and Pathergy negative patients). There was no association between positive Pathergy test and age of patients, or disease duration. The PR was associated with male gender (p = 0.013), oral aphthosis (p < 0.001), pseudofolliculitis (p < 0.001), anterior uveitis (p = 0.001) and posterior uveitis (p = 0.028). Conclusions : The presence of PR was associated with male gender, as well as some of mucocutaneous manifestations and uveitis in adult patients. There was no association with retinitis and vascular involvements. PR isn’t associated with the severity of the disease

  • Classification and Diagnosis Criteria for Behçet’s Disease
    Behçet's Syndrome, 2013
    Co-Authors: Fereydoun Davatchi, Farhad Shahram, Bahar Sadeghi Abdollahi, Cheyda Chams-davatchi, Hormoz Shams, Abdolhadi Nadji
    Abstract:

    Behcet’s disease is relatively a young disease, described in 1937. Nevertheless, it has already 17 sets of diagnosis/classification criteria. The first set was created in 1946 and the last in 2010. The first created by an international collaboration was the International Study Group criteria (ISG) in 1990 with the collaboration of 7 countries, and the last the International Criteria for Behcet’s Disease (ICBD) in 2010 with the collaboration of 27 countries. In ICBD, the following signs get each 2 points: oral aphthosis, genital aphthosis, and ocular manifestations. Skin manifestations, vascular lesions, neurologic manifestations, and a positive Pathergy test get each 1 point. To classify or diagnose a patient as Behcet’s disease, the patient must totalize 4 or more points. As Pathergy test is not performed in some countries, mainly western countries, its use is facultative. However, in patients not fulfilling the ICBD it is recommended to perform it. It is also recommended to classify or diagnose a patient if no other disease could explain the symptoms.

  • impact of the positive Pathergy test on the performance of classification diagnosis criteria for behcet s disease
    Modern Rheumatology, 2013
    Co-Authors: Fereydoun Davatchi, Farhad Shahram, Abdolhadi Nadji, Bahar Sadeghi Abdollahi, Zahra Ghodsi, Massoomeh Akhlaghi, Tahereh Faezi, Hormoz Shams, Cheyda Chamsdavatchi, Roghieh Larimi
    Abstract:

    Background The only diagnostic test that currently exists for Behcet’s disease (BD) is the Pathergy test. A positive Pathergy test (PPT) is an important component of many of the 16 sets of classification/diagnosis criteria used to diagnose BD. The aim of this study was to determine the importance of a PTT in the performance of the diagnosis/classification criteria for BD.

Abdolhadi Nadji - One of the best experts on this subject based on the ideXlab platform.

  • Dermatoscopic and Mucoscopic Features of Lesions in Patients with Behcet's Disease.
    Acta reumatologica portuguesa, 2019
    Co-Authors: Seyedeh Zahra Ghodsi, Farhad Shahram, Fereydoun Davatchi, Abdolhadi Nadji, H. Chams, Bahrololoumi N. Bafruee, Chams C. Davatchi, Cliff Rosendahl, Bengü Nisa Akay, Maassoomeh Akhlaghi
    Abstract:

    Objective: Behcet's disease (BD), as a vasculitis, can affect small and large vessels. As dermatoscopy has been shown to improve the accuracy in diagnosis of various skin lesions especially vascular patterns, we set this study to find if there is any characteristic pattern in the dermatoscopy of Behcet's mucocutaneous lesions.Methods: This prospective cross-sectional observational study designed to evaluate dermatoscopic features of Behcet's mucocutaneous lesions. Fifty six consecutive patients presenting at the outpatient clinic of the BD Research Unit were included. If present, for each patient one oral, one skin and one genital lesion were evaluated by dermatoscopy. When indicated, Pathergy test was performed according to a standard protocol and the results were evaluated by dermatoscopy.Results: A total of 40 oral, 8 genital, 14 skin lesions and 14 Pathergy tests were evaluated by dermatoscopy. While vascular component was the most prominent feature in oral aphthae, this component was less prominent in genital lesions. Dot vessels were the most common form of vessels in both oral and skin lesions. All the oral lesions were characterized by a central white structureless area. Skin lesions were characterized by a red structureless background. In Pathergy tests, negative pricks showed absence of specific features while positive pricks were characterized by a structureless background in pink, purple or red. No obvious vascular component was detected in any of the pricks.Conclusion: It seems that these findings have no specific clues for the diagnosis of BD, but our study is the first study in this field and the findings may give way to further investigations.

  • The association of Pathergy reaction and active clinical presentations of Behçet's disease.
    Reumatologia, 2017
    Co-Authors: Shirin Assar, Farhad Shahram, Bahar Sadeghi, Fereydoun Davatchi, Seyyedeh Zahra Ghodsi, Abdolhadi Nadji, Farimah Ashofte, Seyyedeh Roghieh Larimi, Masoud Sadeghi
    Abstract:

    OBJECTIVES The Pathergy skin test is a hypersensitivity reaction to a prick skin trauma caused by a pin or a needle, which is considered as a specific presentation in Behcet's disease (BD) and the precise mechanism of this test is not well elucidated. This study was designed to evaluate the association of Pathergy reaction (PR) with the active clinical manifestations of BD patients, to assess the clinical importance of PR. MATERIALS AND METHODS This was a cohort study on 1675 BD patients who fulfilled the entry criteria based on the International Criteria for Behcet's Disease (ICBD) from 1975 to 2011. The patients were divided into two groups; the Pathergy positive group included 841 patients (50.2%) and the Pathergy negative group 834 patients (49.8%). The active mucocutaneous and systemic disease manifestations were analyzed according to the presence of the PR. The odds ratio and 95% confidence interval (95% CI) were calculated for each item. RESULTS In this study, 841 patients (50.2%) had a positive Pathergy test. Patient's mean age and mean disease duration were similar in the two groups (Pathergy positive and Pathergy negative patients). There was no association between positive Pathergy test and age of patients, or disease duration. The PR was associated with male gender (p = 0.013), oral aphthosis (p < 0.001), pseudofolliculitis (p < 0.001), anterior uveitis (p = 0.001) and posterior uveitis (p = 0.028). CONCLUSIONS The presence of PR was associated with male gender, as well as some of mucocutaneous manifestations and uveitis in adult patients. There was no association with retinitis and vascular involvements. PR isn't associated with the severity of the disease.

  • The association of Pathergy reaction and active clinical presentations of Behçet’s disease
    Reumatologia Rheumatology, 2017
    Co-Authors: Shirin Assar, Farhad Shahram, Bahar Sadeghi, Fereydoun Davatchi, Seyyedeh Zahra Ghodsi, Abdolhadi Nadji, Farimah Ashofte, Seyyedeh Roghieh Larimi, Masoud Sadeghi
    Abstract:

    Objectives : The Pathergy skin test is a hypersensitivity reaction to a prick skin trauma caused by a pin or a needle, which is considered as a specific presentation in Behçet’s disease (BD) and the precise mechanism of this test is not well elucidated. This study was designed to evaluate the association of Pathergy reaction (PR) with the active clinical manifestations of BD patients, to assess the clinical importance of PR. Materials and methods : This was a cohort study on 1675 BD patients who fulfilled the entry criteria based on the International Criteria for Behçet’s Disease (ICBD) from 1975 to 2011. The patients were divided into two groups; the Pathergy positive group included 841 patients (50.2%) and the Pathergy negative group 834 patients (49.8%). The active mucocutaneous and systemic disease manifestations were analyzed according to the presence of the PR. The odds ratio and 95% confidence interval (95% CI) were calculated for each item. Results : In this study, 841 patients (50.2%) had a positive Pathergy test. Patient’s mean age and mean disease duration were similar in the two groups (Pathergy positive and Pathergy negative patients). There was no association between positive Pathergy test and age of patients, or disease duration. The PR was associated with male gender (p = 0.013), oral aphthosis (p < 0.001), pseudofolliculitis (p < 0.001), anterior uveitis (p = 0.001) and posterior uveitis (p = 0.028). Conclusions : The presence of PR was associated with male gender, as well as some of mucocutaneous manifestations and uveitis in adult patients. There was no association with retinitis and vascular involvements. PR isn’t associated with the severity of the disease

  • Classification and Diagnosis Criteria for Behçet’s Disease
    Behçet's Syndrome, 2013
    Co-Authors: Fereydoun Davatchi, Farhad Shahram, Bahar Sadeghi Abdollahi, Cheyda Chams-davatchi, Hormoz Shams, Abdolhadi Nadji
    Abstract:

    Behcet’s disease is relatively a young disease, described in 1937. Nevertheless, it has already 17 sets of diagnosis/classification criteria. The first set was created in 1946 and the last in 2010. The first created by an international collaboration was the International Study Group criteria (ISG) in 1990 with the collaboration of 7 countries, and the last the International Criteria for Behcet’s Disease (ICBD) in 2010 with the collaboration of 27 countries. In ICBD, the following signs get each 2 points: oral aphthosis, genital aphthosis, and ocular manifestations. Skin manifestations, vascular lesions, neurologic manifestations, and a positive Pathergy test get each 1 point. To classify or diagnose a patient as Behcet’s disease, the patient must totalize 4 or more points. As Pathergy test is not performed in some countries, mainly western countries, its use is facultative. However, in patients not fulfilling the ICBD it is recommended to perform it. It is also recommended to classify or diagnose a patient if no other disease could explain the symptoms.

  • impact of the positive Pathergy test on the performance of classification diagnosis criteria for behcet s disease
    Modern Rheumatology, 2013
    Co-Authors: Fereydoun Davatchi, Farhad Shahram, Abdolhadi Nadji, Bahar Sadeghi Abdollahi, Zahra Ghodsi, Massoomeh Akhlaghi, Tahereh Faezi, Hormoz Shams, Cheyda Chamsdavatchi, Roghieh Larimi
    Abstract:

    Background The only diagnostic test that currently exists for Behcet’s disease (BD) is the Pathergy test. A positive Pathergy test (PPT) is an important component of many of the 16 sets of classification/diagnosis criteria used to diagnose BD. The aim of this study was to determine the importance of a PTT in the performance of the diagnosis/classification criteria for BD.

H Yazici - One of the best experts on this subject based on the ideXlab platform.

  • Behçet Syndrome: Is It One Condition?
    Clinical Reviews in Allergy & Immunology, 2012
    Co-Authors: H Yazici, S. Ugurlu, E. Seyahi
    Abstract:

    Behçet's syndrome (BS) is a disease of unknown etiology, and as such, there have been efforts to classify BS within the popular nosological identities of the times such as seronegative spondarthritides, autoimmune, and more recently autoinflammatory diseases. Current evidence suggests that BS does not easily fit into any one of these lumps, while on occasion, it might be impossible to tell BS from Crohn's disease, especially when the main clinical presentation is intestinal ulceration. There are distinct regional differences in disease expression of BS with fewer cases of intestinal disease in the Mediterranean basin and less severe eye disease and less frequent skin Pathergy among patients reported from northern Europe or America. The clustering of symptoms, especially with the recently described increased frequency of the acne/arthritis cluster in familial cases, suggests that more than one pathological pathway is involved in what we call BS today. Supportive evidence for this contention also comes from the observations that (a) the genetic component is very complex with perhaps different genetic modes of inheritance in the adult and in the pediatric patients; and (b) there are differing organ responses to one same drug. For example, the anti-TNF agents successfully control the oral ulcers while they have no effect on the Pathergy reaction.

  • Frequency of Pathergy phenomenon and other features of Behçet's syndrome among patients with inflammatory bowel disease.
    Clinical and experimental rheumatology, 2008
    Co-Authors: I Hatemi, Melike Melikoglu, Y Ozyazgan, G Hatemi, A F Celik, N Arzuhal, C Mat, H Yazici
    Abstract:

    Crohn's disease (CD) and ulcerative colitis (UC) share common clinical features with Behçet's syndrome (BS). We surveyed UC and CD patients for Pathergy phenomenon and features of BS with the aim of determining how much overlap is present between these 2 entities in a setting where BS is relatively common, the frequency of Pathergy positivity in inflammatory bowel disease (IBD) patients and evaluating how International Study Group (ISG) criteria perform in differentiating IBD from BS. This study was conducted among patients with CD and UC attending the gastroenterology outpatient clinic of a university hospital which is also a referral center for BS. Consecutive CD and UC patients were screened for BS using ISG criteria. Pathergy test was performed and evaluated by 2 independent observers in a masked manner. Ninety-three patients with CD and 130 with UC were surveyed. None of the CD patients fulfilled ISG criteria for BS while 2 of 130 UC patients did. Twenty CD patients had oral ulcers while 4 reported having genital ulcers but no scars could be observed. Twenty-two CD patients had papulopustular lesions, 2 had nodular lesions, 3 had arthritis and none had uveitis. Thirty-two UC patients had oral ulcers, none had genital ulcers, 23 had papulopustular lesions, 3 had nodular lesions, 2 had arthritis and 2 had uveitis. Pathergy test was positive according to at least one of the observers in 10/93 CD and 8/130 UC patients and according to both observers in 4/130 UC patients. Despite similarities between the clinical features of CD and UC with BS, coexistence is uncommon. ISG criteria perform well in differentiating these diseases. About 8% of IBD patients show the Pathergy phenomenon.

  • short term trial of etanercept in behcet s disease a double blind placebo controlled study
    The Journal of Rheumatology, 2005
    Co-Authors: Melike Melikoglu, I Fresko, Y Ozyazgan, Feride Gogus, S Yurdakul, Vedat Hamuryudan, H Yazici
    Abstract:

    OBJECTIVE: To determine the effect of the tumor necrosis factor-alpha blocker etanercept on the Pathergy and monosodium urate (MSU) status and on the mucocutaneous and articular manifestations of patients with Behcet's disease (BD). METHODS: Forty male patients with BD, all with positive Pathergy and MSU tests and mucocutaneous disease and/or arthritis, were randomized (20 patients to each study arm) to receive either etanercept 25 mg twice a week or placebo for 4 weeks. The Pathergy and MSU responses and the frequencies of mucocutaneous and articular manifestations were compared between the 2 groups. RESULTS: There were no decreases in the Pathergy and MSU responses in the etanercept group compared to the placebo group at any time. The mean numbers of oral ulcers, nodular lesions, and papulopustular lesions were less in the etanercept group compared to the placebo group at all weekly evaluations, except for the second week for papulopustular lesions. The probability of being free of oral ulcers and nodular lesions was also significantly higher in the former group (log-rank chi-square = 9.83, p = 0.0017; log-rank chi-square = 14.17, p = 0.0002, respectively). CONCLUSION: Etanercept did not affect the Pathergy reaction and the cutaneous response to MSU crystals. However, the drug was effective in suppressing most of the mucocutaneous manifestations of BD.

  • Short-term trial of etanercept in Behçet's disease: a double blind, placebo controlled study.
    The Journal of rheumatology, 2005
    Co-Authors: Melike Melikoglu, I Fresko, Y Ozyazgan, Feride Gogus, S Yurdakul, Vedat Hamuryudan, Cem Mat, H Yazici
    Abstract:

    OBJECTIVE: To determine the effect of the tumor necrosis factor-alpha blocker etanercept on the Pathergy and monosodium urate (MSU) status and on the mucocutaneous and articular manifestations of patients with Behcet's disease (BD). METHODS: Forty male patients with BD, all with positive Pathergy and MSU tests and mucocutaneous disease and/or arthritis, were randomized (20 patients to each study arm) to receive either etanercept 25 mg twice a week or placebo for 4 weeks. The Pathergy and MSU responses and the frequencies of mucocutaneous and articular manifestations were compared between the 2 groups. RESULTS: There were no decreases in the Pathergy and MSU responses in the etanercept group compared to the placebo group at any time. The mean numbers of oral ulcers, nodular lesions, and papulopustular lesions were less in the etanercept group compared to the placebo group at all weekly evaluations, except for the second week for papulopustular lesions. The probability of being free of oral ulcers and nodular lesions was also significantly higher in the former group (log-rank chi-square = 9.83, p = 0.0017; log-rank chi-square = 14.17, p = 0.0002, respectively). CONCLUSION: Etanercept did not affect the Pathergy reaction and the cutaneous response to MSU crystals. However, the drug was effective in suppressing most of the mucocutaneous manifestations of BD.

A Gul - One of the best experts on this subject based on the ideXlab platform.

  • immunohistology of skin Pathergy reaction in behcet s disease
    British Journal of Dermatology, 2010
    Co-Authors: A Gul, Semih Esin, N Dilsen, M Konice, Hans Wigzell, P Biberfeld
    Abstract:

    The immunophenotypic characteristics of the skin Pathergy reaction (SPR) at 48h in Behcet's disease (BD) were investigated in 12 patients with BD and in five controls. The findings in 11 positive and one negative SPR lesions of patients with BD were evaluated in comparison with those of normal adjacent skin and with the negative Pathergy biopsies from the controls. Positive SPR biopsies showed variable epidermal thickening and cell vacuolization, as well as subcorneal pustule formation. In the SPR dermis, a variable dense focal mononuclear cell (MNC) infiltrate was seen around vessels and skin appendages, extending into the deep dermis. The MNC infiltrate was predominantly composed of T lymphocytes and monocytes/macrophages. The majority of the T lymphocytes were CD4 + , and almost all expressed CD45RO. Approximately half of the infiltrating ceils strongly expressed HLA-DR. Neutrophils constituted less than 5% of the infiltrating cells, but were present as clusters of elastase-positive cells at the needle-prick sites. Vessels within the lesion showed marked congestion and endothelial swelling. The endothelial cells expressed ICAM-1 strongly, and E-selectin moderately. VCAM-1 was not expressed on endothelial cells. The basal and mid-epidermal layers of keratinocytes expressed HLA-DR and ICAM-1 strongly, particularly so in areas close to the dermal MNC infiltrates. In negative Pathergy biopsies, there were increased numbers of neutrophils and a few small clusters of macrophages and T lymphocytes only at the needle-prick site, and the endothelial cells of vessels close to these areas expressed E-selectin weakly. The immunohistological findings of the SPR appear to indicate an augmented antigen-independent non-specific induction phase of the inflammatory response. Absence of VCAM-1 expression by endothelial cells suggests that direct epidermal injury is the cause of the cutaneous inflammation.