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Pathergy

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101. Postoperative pyoderma gangrenosum: diagnostic value of 16s ribosomal RNA sequencing and review of the literature. (PubMed)

Postoperative pyoderma gangrenosum: diagnostic value of 16s ribosomal RNA sequencing and review of the literature. Pyoderma gangrenosum (PG) is an uncommon condition, but can lead to the diagnosis of an underlying systemic disease. It can appear spontaneously or as a result of the pathergy phenomenon after trauma or surgery.We review three patients with postoperative PG (PPG) and the relevant literature. We also report an accurate method for microbial detection by 16S ribosomal (r)RNA

2008 Clinical & Experimental Dermatology

102. Traumatic Pyoderma Gangrenosum of the Face: Pathergy Development after Bike Accident. (PubMed)

Traumatic Pyoderma Gangrenosum of the Face: Pathergy Development after Bike Accident. We present a patient who developed pyoderma gangrenosum (PG) twice, initially after a minor trauma and later after reconstructive surgery. This case is presented to address the frequent misdiagnosis and mistreatment of PG by surgeons. It is of great importance that PG is diagnosed and of even great importance that surgery is avoided in order to prevent pathergy. Our case adds to the understanding

2008 Dermatology

103. Pyoderma gangrenosum following isotretinoin therapy for acne nodulocystic. (PubMed)

Pyoderma gangrenosum following isotretinoin therapy for acne nodulocystic. A 19-year-old man with nodulocystic acne on baseline was treated with isotretinoin therapy. After 1 month on the medication, he developed pyoderma gangrenosum on his pubis area, arms and legs, and pathergy on a puncture site. Possible underlying diseases were excluded. The patient was started on steroids (prednisone 1 mg/kg/d) and isotretinoin therapy was withdrawn. Later the prednisone was tapered and dapsone 100 mg

2008 International Journal of Dermatology

104. Pyoderma Gangrenosum

per million population per year worldwide. [ ] A UK study estimated incidence rate to be around 0.91 per 100,000 person-years. [ ] It may occur at any age, but the peak incidence is over the age of 50, with a slight female preponderance. Aetiology The cause is unknown. 50-70% of cases are associated with other diseases, mainly inflammatory bowel disease (IBD), arthritis and lymphoproliferative disorders. [ ] PG may occur in sites of trauma; this phenomenon is called pathergy . It often begins (...) arthritis, pyoderma gangrenosum and acne syndrome (PAPA syndrome). [ ] Other conditions - also reported with paroxysmal nocturnal haemoglobinuria, systemic lupus erythematosus, antiphospholipid syndrome, vasculitis and Wegener's granulomatosis. [ ] The occurrence of PG does not seem to relate to the disease activity in conditions such as IBD and arthritis. Possible precipitating factors for PG are : [ , ] Biopsies, intradermal skin testing, injections, insect bites, etc (due to pathergy). Surgery

2008 Mentor

105. Behçet's Disease

lamp examination or retinal vasculitis observed by an ophthalmologist. Skin lesions: erythema nodosum, pseudo-folliculitis, papulopustular lesions or acneiform nodules in post-adolescent patients not on corticosteroids. Pathergy (exaggerated skin injury occurring after minor trauma), read by a physician at 24-48 hours. Epidemiology [ ] This condition is not commonly seen in Northern Europeans. The prevalence is highest in the Middle East, Mediterranean and Eastern Asia. [ ] The prevalence is 80 (...) possible differential diagnoses and to assess the presenting features that may be present: FBC may show mild anaemia and raised white cell count. Nonspecific inflammatory markers: CRP, ESR, complement and acute-phase reactants may all be elevated during an acute attack. Antiphospholipid antibodies are positive in 25% of patients. Pathergy test: minor skin trauma induces an inflammatory papule or pustule after 24-48 hours. This is positive in up to 60% of patients. Imaging studies, including CT

2008 Mentor

106. Behcet disease

-a, IL-8, IL-12, IL-17 and IL-21) have been implicated in the pathogenesis of BD. Diagnostic methods International classification criteria, as defined by clinical presentation, are sensitive and specific. The presence of recurrent oral aphtae, at least 3 times over 12 months, is mandatory, in combination with two of the following: recurrent genital ulceration, eye lesions, skin lesions or pathergy. In neuro-BD, lumbar puncture is mandatory and MRI may reveal inflammatory lesions in the cerebral trunk

2005 Orphanet

107. Pyoderma Gangrenosum and Full-Thickness Burns: is there a Problem of Differential Diagnosis? (PubMed)

Pyoderma Gangrenosum and Full-Thickness Burns: is there a Problem of Differential Diagnosis? A case is presented of pyoderma gangrenosum, describing its characteristic features with particular reference to the phenomenon of pathergy, which considerably limits the surgical approach to the disease. After an account of the numerous skin ulcerations that have to be taken into consideration in order to make a differential diagnosis, it is noted that pyoderma gangrenosum can only on rare occasions

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2006 Annals of burns and fire disasters

108. Short-term trial of etanercept in Behçet's disease: a double blind, placebo controlled study. (PubMed)

Short-term trial of etanercept in Behçet's disease: a double blind, placebo controlled study. 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 Behçet's disease (BD).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.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

2005 The Journal of rheumatology

109. Involvement of chemokines and Th1 cytokines in the pathogenesis of mucocutaneous lesions of Behçet's disease. (PubMed)

at the site of pathergy testing were studied using real-time quantitative reverse transcriptase-polymerase chain reaction.We observed important increases in the expression of interleukin-8 (IL-8) ( approximately 700-fold), monocyte chemoattractant protein 1 ( approximately 65-fold), interferon-gamma ( approximately 71-fold), and IL-12 ( approximately 69-fold) messenger RNA in BD lesions compared with normal skin. Except for IL-10 ( approximately 75-fold increase), Th2 cytokines (i.e., IL-4 and IL-13) were

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2004 Arthritis and Rheumatism

110. Clinical features and natural course of Behçet's disease in 661 cases: a multicentre study. (PubMed)

hundred and sixty-one patients were involved in this multicentre study. The symptoms of the disease were recorded retrospectively in the time order of the manifestations in each patient.Oral ulcers were the most common manifestation (100%), followed by genital ulcers (85.3%), papulopustular lesions (55.4%), erythema nodosum (44.2%), skin pathergy reaction (37.8%), and articular (33.4%) and ocular involvement (29.2%). Oral ulcers were the most common onset manifestation (88.7%). The mean +/- SD (...) duration between the onset symptom and the fulfilment of diagnostic criteria was calculated to be 4.3 +/- 5.7 years. The clinical severity score was significantly increased in the noncompliant treatment group compared with the compliant group with the passage of time (P < 0.001). The frequency of ocular involvement and genital ulcers was significantly higher in patients whose disease onset was at < 40 years. Genital ulcers, ocular involvement, papulopustular lesions, thrombophlebitis and skin pathergy

2007 British Journal of Dermatology

111. Clinical features of patients with Behçet's disease and epididymitis. (PubMed)

ulcer in 1 (2.8%) and a positive pathergy test in 4 (11.1%). A significantly higher number of patients with epididymitis had genital ulcers (p <0.05), cutaneous involvement (p <0.001), arthritis (p <0.05), central nervous system involvement (p <0.05) and a positive pathergy test (p <0.05) compared with the other 744 with Behçet's disease without epididymitis who served as controls.To our knowledge there has been no controlled study of the clinical significance of epididymitis in Behçet's disease

2003 Journal of Urology

112. Serum lipoprotein (a) levels and Behçet's disease: is there an association? (PubMed)

eye involvement. Twelve (30%) had a positive pathergy test. Four (10%) had vascular involvement. The Lp(a) level of the patient population was 19.6+/-18.8 mg/dL. This level was higher than that of the controls, but not statistically significant. The Lp(a) levels of the four patients with vascular complications were within normal limits.Lp(a) is of interest, as it is a genetically determined parameter that was found to be high in BD patients in our study group. The levels were independent

2007 International Journal of Dermatology

113. Behçet's disease-like presentation of bullous pyoderma gangrenosum associated with Crohn's disease. (PubMed)

Behçet's disease-like presentation of bullous pyoderma gangrenosum associated with Crohn's disease. A 47-year-old woman presented with a 2-month history of generalized arthralgia and a 10-day history of oral aphthous ulcers. After hospitalization, papulopustular lesions and perianal ulcerations developed. Pathergy test was positive and ophthalmological examination was normal. The presence of oral aphthous ulcers, genital ulcerations, papulopustular lesions and arthralgia, and the positive (...) pathergy test suggested the diagnosis of Behçet's disease (BD). In a few days, positive pathergy reactions and papulopustular lesions evolved into bullous lesions, which were diagnosed dermatopathologically as pyoderma gangrenosum. Two days after the presentation of papulopustular lesions, the patient experienced diarrhoea accompanied by bloody stools and mucus. Histopathological examination of biopsy specimens showed no vasculitis but revealed findings suggestive of Crohn's disease. The patient

2006 Clinical & Experimental Dermatology

114. Cavitary pyoderma gangrenosum treated with local infusion of corticosteroid. (PubMed)

Cavitary pyoderma gangrenosum treated with local infusion of corticosteroid. Pyoderma gangrenosum (PG) is a rare inflammatory skin condition, characterized by progressive and recurrent skin ulceration, often representing a pathergy response.We report a case of spontaneous retrosternal PG that developed on the background of previous PG of the skin of the back. The patient had underlying ulcerative colitis. After failure of prednisone, minocycline, and dapsone, a novel method was instituted

2007 Journal of American Academy of Dermatology

115. TNF-alpha gene 1031 T/C polymorphism in Turkish patients with Behçet's disease. (PubMed)

of TNF-alpha-1031C [P = 0.018, OR = 1.83, 95% confidence interval (CI) = 1.07-3.13]. When the allele frequencies were analysed according to the clinical features, the T allele in patients with positive skin pathergy test (SPT) was significantly increased when compared with those of patients without these findings (P = 0.004, OR = 2.75, 95% CI = 1.3-5.86). To demonstrate the frequency of TNF-alpha and IFN-gamma producing cells, mononuclear cells from four representative individuals of each genotype (...) in the spontaneous IFN-gamma values between groups were not statistically significant, the stimulated IFN-gamma values were found to be significantly increased in the BD group when compared with the healthy control group (P = 0.004).Our results showed that, in the Turkish population the TNF-alpha-1031C allele is associated with susceptibility to BD. On the other hand, carrying the T allele may render patients more prone to developing a positive skin pathergy test. In addition, ELISPOT assays revealed that BD

2006 British Journal of Dermatology

116. Successful use of vacuum-assisted closure therapy for leg ulcers caused by occluding vasculopathy and inflammatory vascular diseases--a case series. (PubMed)

disease, who were treated successfully using vacuum-assisted closure (VAC) for wound management. We present the advantages and disadvantages of this method, as well as illustrating the essential and known therapeutic principles.Our experience shows VAC to be an excellent and effective alternative in the treatment of therapy-resistant chronic wounds caused by vasculopathy (small vessel occlusion or vasculitis). We did not observe any pathergy or proinflammatory effects caused by VAC.2007 S. Karger AG

2007 Dermatology

117. Clinical features of late-onset Behçet's disease: report of nine cases. (PubMed)

(RAS) until 50 years of age but fulfilled the diagnostic criteria of BD after this age were reviewed.The age-of-onset was more than 50 years in nine patients (1.56%). Two patients developed erythema nodosum, two developed pathergy positivity, one developed papulopustules, pathergy positivity and ocular symptoms, one developed papulopustules and pathergy positivity, one developed ocular symptoms, one developed papulopustules and ocular symptoms, and one developed erythema nodosum and pathergy

2006 International Journal of Dermatology

118. Clinical features of Behçet's disease in children. (PubMed)

%), ocular lesions in 4 (24%), erythema nodosum in 3 (18%), papulopustules in 8 (47%), joint symptoms in 13 (76%), mild gastrointestinal symptoms in 2 (12%), neurological involvement in 2 (12%), positive pathergy reactions in 13 (76%), and thrombophlebitis in 1 (6%). We used colchicine, systemic corticosteroids, cyclosporine, nonsteroidal anti-inflammatory drugs, sulfasalazine, and topical agents for the treatment of children with BD.The findings of BD in children are similar as in adults

2006 International Journal of Dermatology

119. Pyoderma gangrenosum: an update. (PubMed)

Pyoderma gangrenosum: an update. Pyoderma gangrenosum (PG) can be differentiated into classic and atypical forms. The classic form is characterized by ulcers and the atypical form by deep erosions with bullous blue-gray margins. Pathergy, the development of cutaneous lesions at sites of trauma, is a common feature of both forms of PG. Approximately 50% of patients who have PG have underlying systemic diseases, most commonly inflammatory bowel disease, myeloproliferative disorders, and various

2007 Rheumatic Diseases Clinics of North America

120. Behçet's disease associated with amyloidosis in Turkey and in the world. (PubMed)

Behçet's disease associated with amyloidosis in Turkey and in the world. The association of amyloidosis with Behçet's disease has infrequently been reported in published works. Twenty four such cases have been observed in the world, of which 12 are from Turkey, including eight of ours. In all our eight cases renal biopsy showed amyloidosis of type AA. Behçet's disease of male preponderance, long duration, complete type, multiple organ involvement, and positive skin pathergy test were the main

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1988 Annals of the Rheumatic Diseases

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