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Pathergy

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61. 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

62. Subcorneal Pustular Dermatosis (Treatment)

, 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

63. Behcet Disease (Treatment)

Spontaneous arterial aneurysm formation Thrombotic obstruction in large-caliber vessels Cardiac involvement Proper timing for surgical treatment is important. Delayed wound healing or inflammation at operative sites may be related to pathergy. Previous Next: Consultations Consultation with the following specialists may be necessary: Dermatologist - For evaluation of mucocutaneous lesions (ie, oral ulcer, genital ulcer, skin lesions) Ophthalmologist - For evaluation of eye involvement Rheumatologist (...) 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):89-94. . Akar S, Ozcan MA, Ates H, Gürler

2014 eMedicine.com

64. Pyoderma Gangrenosum (Treatment)

conditions. Surgery Surgery should be avoided, if possible, because of the pathergic phenomenon that may occur with surgical manipulation or grafting, resulting in wound enlargement. [ ] Pathergy is seen in about 30% of cases. In some patients, grafting has resulted in the development of pyoderma gangrenosum at the harvest site. In cases in which surgery is required, the best plan, if possible, is to have the patient on therapy, and active disease under control, in order to prevent pathergy. Some

2014 eMedicine.com

65. Pyoderma Gangrenosum (Overview)

diseases, diabetes, and trauma. In a process termed pathergy, new ulcerations may occur after trauma or injury to the skin in 30% of patients who already have pyoderma gangrenosum. (See , , and .) Patients with pyoderma gangrenosum may have involvement of other organ systems that manifests as sterile neutrophilic infiltrates. Culture-negative pulmonary infiltrates are the most common extracutaneous manifestation. [ , ] Other organs systems that may be involved include the heart, the central nervous (...) fail. These patients pose a difficult clinical problem that requires frequent follow-up and long-term care. Some patients demonstrate pathergy, or the development of pyoderma gangrenosum–like lesions at the site of skin trauma; in such instances, protection of the skin from trauma may prevent a recurrence of the disease. Pathergy may create problems with wound healing, especially after surgical procedures (eg, breast reconstruction). [ , ] Death from pyoderma gangrenosum is rare, but it may occur

2014 eMedicine.com

66. 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

67. 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

68. 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

69. Subcorneal Pustular Dermatosis (Overview)

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

70. Pyoderma Gangrenosum (Follow-up)

conditions. Surgery Surgery should be avoided, if possible, because of the pathergic phenomenon that may occur with surgical manipulation or grafting, resulting in wound enlargement. [ ] Pathergy is seen in about 30% of cases. In some patients, grafting has resulted in the development of pyoderma gangrenosum at the harvest site. In cases in which surgery is required, the best plan, if possible, is to have the patient on therapy, and active disease under control, in order to prevent pathergy. Some

2014 eMedicine.com

71. 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

72. 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

73. 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

74. 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

75. 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

76. 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

77. Pyoderma Gangrenosum (Diagnosis)

diseases, diabetes, and trauma. In a process termed pathergy, new ulcerations may occur after trauma or injury to the skin in 30% of patients who already have pyoderma gangrenosum. (See , , and .) Patients with pyoderma gangrenosum may have involvement of other organ systems that manifests as sterile neutrophilic infiltrates. Culture-negative pulmonary infiltrates are the most common extracutaneous manifestation. [ , ] Other organs systems that may be involved include the heart, the central nervous (...) fail. These patients pose a difficult clinical problem that requires frequent follow-up and long-term care. Some patients demonstrate pathergy, or the development of pyoderma gangrenosum–like lesions at the site of skin trauma; in such instances, protection of the skin from trauma may prevent a recurrence of the disease. Pathergy may create problems with wound healing, especially after surgical procedures (eg, breast reconstruction). [ , ] Death from pyoderma gangrenosum is rare, but it may occur

2014 eMedicine.com

78. 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

79. 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

80. 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

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