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61. Behcet Disease (Overview)

given to ocular disease. In 1990, The International Study Group (ISG) for Behçet's Disease proposed a separate set of diagnostic criteria for Behçet disease. Based on these criteria, a diagnosis of Behçet disease requires recurrent oral ulceration and at least 2 additional criteria, including recurrent genital ulcers, ocular lesions, skin lesions, and a positive pathergy test. The ISG criteria for Behçet disease have excellent specificity, but lack sensitivity. The International Criteria (...) in the frequency of a positive pathergy reaction with surgical cleaning of the skin before the procedure, the acne-arthritis association carrying similar features to acne-associated reactive arthritis, a higher rate of tonsillectomy, cold sores, late birth order, higher number of siblings, history of travel to countries with a high incidence of Behçet syndrome, and earlier age at first sexual intercourse. Moreover, basic research on both viruses and bacteria suggests that microorganisms may be playing a role

2014 eMedicine.com

62. Behcet Disease (Overview)

, pustular lesions, and pathergy reactions), the activity and function of these cells has been explored extensively. Unfortunately, existing studies offer inconsistent results regarding cell adhesion and chemotactic behavior, superoxide production, and phagocytic properties. Thus, the specific role of neutrophils in Behçet disease has been difficult to characterize. Some studies have found that cytokine release in Behçet disease may, by an unknown mechanism, place neutrophils in a static pre-excitatory

2014 eMedicine.com

63. Behcet Disease (Overview)

is a receptor on vascular endothelial cells, which, when down-regulated, leads to a procoagulation state. High levels of thrombomodulin are mostly observed in persons with skin pathergy reactions. [ ] Decreased levels of antithrombotic factors, including fibrinolysis and activated protein C, are noted. [ ] Additional factors include decreased deformability of red blood cells [ ] and increased platelet activity. [ ] However, thrombophilia in Behçet disease patients does not seem to be the major factor (...) . Strong association between plasma thrombomodulin and pathergy test in Behcet disease. Ann Rheum Dis . 2008 Jun. 67(6):892-3. . Espinosa G, Font J, Tàssies D, Vidaller A, Deulofeu R, López-Soto A, et al. Vascular involvement in Behçet's disease: relation with thrombophilic factors, coagulation activation, and thrombomodulin. Am J Med . 2002 Jan. 112(1):37-43. . Usküdar O, Erdem A, Demiroglu H, Dikmenoglu N. Decreased erythrocyte deformability in Behçet's disease. Clin Hemorheol Microcirc . 2005. 33(2

2014 eMedicine.com

64. Subcorneal Pustular Dermatosis (Follow-up)

, Schwartz RA, Lambert WC. Subcorneal pustular dermatosis with polyarthritis. Cutis . 1986 Feb. 37(2):123-4, 126. . Delaporte E, Colombel JF, Nguyen-Mailfer C, Piette F, Cortot A, Bergoend H. Subcorneal pustular dermatosis in a patient with Crohn's disease. Acta Derm Venereol . 1992 Aug. 72 (4):301-2. . García-Salces I, Baldellou R, Hörndler C, Zubiri ML. Subcorneal pustular dermatosis with pathergy phenomenon in a patient with Crohn's disease. J Eur Acad Dermatol Venereol . 2009 Mar. 23 (3):349-50

2014 eMedicine.com

65. Subcorneal Pustular Dermatosis (Diagnosis)

in association with rheumatoid arthritis. Br J Dermatol . 1995 Feb. 132(2):313-5. . Lin RY, Schwartz RA, Lambert WC. Subcorneal pustular dermatosis with polyarthritis. Cutis . 1986 Feb. 37(2):123-4, 126. . Delaporte E, Colombel JF, Nguyen-Mailfer C, Piette F, Cortot A, Bergoend H. Subcorneal pustular dermatosis in a patient with Crohn's disease. Acta Derm Venereol . 1992 Aug. 72 (4):301-2. . García-Salces I, Baldellou R, Hörndler C, Zubiri ML. Subcorneal pustular dermatosis with pathergy phenomenon

2014 eMedicine.com

66. Behcet Disease (Diagnosis)

is a receptor on vascular endothelial cells, which, when down-regulated, leads to a procoagulation state. High levels of thrombomodulin are mostly observed in persons with skin pathergy reactions. [ ] Decreased levels of antithrombotic factors, including fibrinolysis and activated protein C, are noted. [ ] Additional factors include decreased deformability of red blood cells [ ] and increased platelet activity. [ ] However, thrombophilia in Behçet disease patients does not seem to be the major factor (...) . Strong association between plasma thrombomodulin and pathergy test in Behcet disease. Ann Rheum Dis . 2008 Jun. 67(6):892-3. . Espinosa G, Font J, Tàssies D, Vidaller A, Deulofeu R, López-Soto A, et al. Vascular involvement in Behçet's disease: relation with thrombophilic factors, coagulation activation, and thrombomodulin. Am J Med . 2002 Jan. 112(1):37-43. . Usküdar O, Erdem A, Demiroglu H, Dikmenoglu N. Decreased erythrocyte deformability in Behçet's disease. Clin Hemorheol Microcirc . 2005. 33(2

2014 eMedicine.com

67. Behcet Disease (Diagnosis)

given to ocular disease. In 1990, The International Study Group (ISG) for Behçet's Disease proposed a separate set of diagnostic criteria for Behçet disease. Based on these criteria, a diagnosis of Behçet disease requires recurrent oral ulceration and at least 2 additional criteria, including recurrent genital ulcers, ocular lesions, skin lesions, and a positive pathergy test. The ISG criteria for Behçet disease have excellent specificity, but lack sensitivity. The International Criteria (...) in the frequency of a positive pathergy reaction with surgical cleaning of the skin before the procedure, the acne-arthritis association carrying similar features to acne-associated reactive arthritis, a higher rate of tonsillectomy, cold sores, late birth order, higher number of siblings, history of travel to countries with a high incidence of Behçet syndrome, and earlier age at first sexual intercourse. Moreover, basic research on both viruses and bacteria suggests that microorganisms may be playing a role

2014 eMedicine.com

68. Behcet Disease (Diagnosis)

, pustular lesions, and pathergy reactions), the activity and function of these cells has been explored extensively. Unfortunately, existing studies offer inconsistent results regarding cell adhesion and chemotactic behavior, superoxide production, and phagocytic properties. Thus, the specific role of neutrophils in Behçet disease has been difficult to characterize. Some studies have found that cytokine release in Behçet disease may, by an unknown mechanism, place neutrophils in a static pre-excitatory

2014 eMedicine.com

69. Hereditary Periodic Fever Syndromes (Follow-up)

arthritis in early childhood. Occasionally, patients with PAPA syndrome develop significant joint destruction. Dermatological manifestations are also episodic and recurrent and are characterized by debilitating aggressive ulcerative skin lesions, often of the lower extremities. The other component of this triad is cystic acne, which begins in adolescence and persists into adulthood. Other possible manifestations of PAPA syndrome include pathergy (formation of sterile abscesses at injection sites

2014 eMedicine Pediatrics

70. Vasculitis and Thrombophlebitis (Overview)

hypocomplementemia or as part of a disease associated with low complement levels (eg, systemic lupus erythematosus). Other vasculitides involves vessels of all sizes. The diagnosis is made clinically in patients with recurrent oral ulcers who also have recurrent genital ulcers, uveitis, various skin lesions, and/or a positive pathergy test. /Goodpasture syndrome is a type of vasculitis caused by deposition of anti-GBM antibodies in small vessels of lungs and kidneys. Goodpasture syndrome clinically manifests

2014 eMedicine Pediatrics

71. Behcet Syndrome (Diagnosis)

injuries may be superimposed on the hypercoagulability observed in some patients. Neutrophilic hyperfunction is observed in patients with Behçet syndrome with neutrophilic infiltration of skin at the site of a prick with a sterile needle (the pathergy test). Lymphocyte function has also been reported as abnormal, with a clonal expansion of autoreactive T cells. Results of recent genome-wide association studies (GWASs) confirm the association with HLA-B51, although there is currently not a diagnostic (...) . An increased incidence of skin pathergy and HLA-B51 antigen is observed in Middle Eastern and Asian patients, compared with North American or northern European patients. Sex In Japan and Korea, Behçet syndrome is more common in females, with a male-to-female ratio of 1:2, but it is more common in males in the Middle East, with a male-to-female ratio of 2:1. In the literature, estimates of male-to-female ratios range from 11:1 to 0.2:1. Despite the variability of the reported sex ratios, the disease tends

2014 eMedicine Pediatrics

72. Vasculitis and Thrombophlebitis (Diagnosis)

hypocomplementemia or as part of a disease associated with low complement levels (eg, systemic lupus erythematosus). Other vasculitides involves vessels of all sizes. The diagnosis is made clinically in patients with recurrent oral ulcers who also have recurrent genital ulcers, uveitis, various skin lesions, and/or a positive pathergy test. /Goodpasture syndrome is a type of vasculitis caused by deposition of anti-GBM antibodies in small vessels of lungs and kidneys. Goodpasture syndrome clinically manifests

2014 eMedicine Pediatrics

73. Hereditary Periodic Fever Syndromes (Treatment)

arthritis in early childhood. Occasionally, patients with PAPA syndrome develop significant joint destruction. Dermatological manifestations are also episodic and recurrent and are characterized by debilitating aggressive ulcerative skin lesions, often of the lower extremities. The other component of this triad is cystic acne, which begins in adolescence and persists into adulthood. Other possible manifestations of PAPA syndrome include pathergy (formation of sterile abscesses at injection sites

2014 eMedicine Pediatrics

74. Hereditary Periodic Fever Syndromes (Overview)

arthritis in early childhood. Occasionally, patients with PAPA syndrome develop significant joint destruction. Dermatological manifestations are also episodic and recurrent and are characterized by debilitating aggressive ulcerative skin lesions, often of the lower extremities. The other component of this triad is cystic acne, which begins in adolescence and persists into adulthood. Other possible manifestations of PAPA syndrome include pathergy (formation of sterile abscesses at injection sites

2014 eMedicine Pediatrics

75. Behcet Syndrome (Overview)

injuries may be superimposed on the hypercoagulability observed in some patients. Neutrophilic hyperfunction is observed in patients with Behçet syndrome with neutrophilic infiltration of skin at the site of a prick with a sterile needle (the pathergy test). Lymphocyte function has also been reported as abnormal, with a clonal expansion of autoreactive T cells. Results of recent genome-wide association studies (GWASs) confirm the association with HLA-B51, although there is currently not a diagnostic (...) . An increased incidence of skin pathergy and HLA-B51 antigen is observed in Middle Eastern and Asian patients, compared with North American or northern European patients. Sex In Japan and Korea, Behçet syndrome is more common in females, with a male-to-female ratio of 1:2, but it is more common in males in the Middle East, with a male-to-female ratio of 2:1. In the literature, estimates of male-to-female ratios range from 11:1 to 0.2:1. Despite the variability of the reported sex ratios, the disease tends

2014 eMedicine Pediatrics

76. Hereditary Periodic Fever Syndromes (Diagnosis)

arthritis in early childhood. Occasionally, patients with PAPA syndrome develop significant joint destruction. Dermatological manifestations are also episodic and recurrent and are characterized by debilitating aggressive ulcerative skin lesions, often of the lower extremities. The other component of this triad is cystic acne, which begins in adolescence and persists into adulthood. Other possible manifestations of PAPA syndrome include pathergy (formation of sterile abscesses at injection sites

2014 eMedicine Pediatrics

77. Pyoderma gangrenosum in a patient with common variable primary immunodeficiency Full Text available with Trip Pro

-reactive inflammatory response to various factors (presence of the so-called pathergy symptom). Common variable primary immunodeficiency (CVID) is a disease that is rather often recognized and affects about 1/10,000-100,000 individuals. It is a heterogeneous group of disorders of combined B-and T-cell dysfunction. The case is described of a 22-year-old man with pyoderma gangrenosum that coexisted with CVID.

2013 Advances in Dermatology and Allergology/Postȩpy Dermatologii i Alergologii

78. Demographic and clinical features of 521 Turkish patients with Behçet's disease. (Abstract)

) (0.2%). In 120 patients (23%), the onset manifestation compromised more than one symptom. During follow-up, in females and males respectively, oral ulceration was found in 100%, genital ulceration in 90.9% and in 82.5%, papulopustular lesions in 52.6% and in 71.4%, positive pathergy test in 45.3% and in 48.7%, ENLL in 43.6% and in 31.6%, ocular involvement in 36.9% and in 58.1%, gastrointestinal involvement in 6.6% and in 5.6%, joint involvement in 4.2% and in 6.4%, vascular involvement in 1.7

2013 International Journal of Dermatology

79. Pyoderma Gangrenosum

Aka: Pyoderma Gangrenosum , Pathergy II. Epidemiology Rare III. Pathophysiology ic dermatosis Also occurs in Behcet Disease and s release elastase and other enzymes that result in skin breakdown IV. Symptoms Painful s and ulcers that form on the legs, buttocks, and face in response to minor V. Signs Lesion characteristics Initial Hemorrhagic, red or painful, tender Next Lesion ulcerates Ulcer has irregular, raised, undermined border and advances peripherally Ulcer base with exudate (...) that is pustular and hemorrhagic Necrotic eschar may form Next Ulcer heals with thin, atrophic scar Distribution (in order of most common to least common) Legs Buttocks Face Pathergy See ing and ulceration in response to minor Also seen with Behcet Disease VI. Associated Conditions (e.g. , ) (e.g. , ) Hepatitis VII. Differential Diagnosis See VIII. References Fitzpatrick (1992) Color Atlas and Synopsis of Clinical Dermatology Jhun and DeClerck in Herbert (2015) EM:Rap 15(8): 19-20 Images: Related links

2015 FP Notebook

80. Contribution of Hla-B51 in the susceptibility and specific clinical features of Behcet's disease in Tunisian patients. (Abstract)

for the BD classification recruited from the Department of Internal Medicine, Rabta Hospital in Tunis and compared with 125 native Tunisian healthy age and sex matching volunteers.According to our findings, the frequency of HLAB 51 was significantly higher in BD patients than in controls (p<0.001). Positive pathergy test (PPT) (p = 0.01) and retinal vasculitis (p = 0.045), were significantly more frequent in HLA B51(+) patients, while the frequency of arterial aneurysms (p = 0.009) and neurological

2012 European journal of internal medicine

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