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101. Pyoderma gangrenosum associated with subcorneal pustular dermatosis and IgA myeloma. (Abstract)

Pyoderma gangrenosum associated with subcorneal pustular dermatosis and IgA myeloma. We report a 57-year-old woman with a 12-year history of ulcerative pyoderma gangrenosum (PG). Five years after the onset of PG, she developed subcorneal pustular dermatosis (SPD) and biclonal IgA and IgG gammopathy. She developed PG at two bone-marrow biopsy sites, showing pathergy. Finally, she developed multiple myeloma. Although PG and SPD may occur without associated underlying malignancy, these patients

2008 Clinical & Experimental Dermatology

102. Cavitary pyoderma gangrenosum treated with local infusion of corticosteroid. (Abstract)

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

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

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

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

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

105. Clinical features of Behçet's disease in children. (Abstract)

%), 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

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

(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

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

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

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

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

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

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

2004 Arthritis and Rheumatism

110. 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

111. 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

112. 'Sticky' neutrophils, pathergic arthritis, and response to heparin in pyoderma gangrenosum complicating ulcerative colitis. Full Text available with Trip Pro

'Sticky' neutrophils, pathergic arthritis, and response to heparin in pyoderma gangrenosum complicating ulcerative colitis. Pyoderma gangrenosum is strongly associated with inflammatory bowel disease and exhibits pathergy, occurring at sites of previous minor trauma. A patient is presented with a 21 year history of extensive ulcerative colitis, who developed pyoderma gangrenosum and arthralgia while receiving high dose corticosteroids for active ulcerative colitis. The arthralgia exhibited (...) pathergy affecting particularly the left temporomandibular joint, which was stressed by an asymmetric bite, and the left elbow, which had been fractured many years previously. This prompted the hypothesis that neutrophils in this condition may be marginated, as a result of increased stickiness of either the neutrophil or the vascular endothelium. The introduction of heparin therapy was associated with rapid resolution of the arthralgia, pyoderma gangrenosum, and ulcerative colitis.

1995 Gut

113. Response to intradermal injection of monosodium urate crystals in Behçet's syndrome. Full Text available with Trip Pro

Response to intradermal injection of monosodium urate crystals in Behçet's syndrome. The cutaneous response to intradermal injection of monosodium urate crystals was investigated in 97 patients with Behçet's syndrome in Turkey and 14 in the United Kingdom, and in 82 healthy and 88 diseased controls. Urate crystals produced an increased erythematous response in patients compared with controls in both countries. This response was different from that of the pathergy test performed at the same

1991 Annals of the Rheumatic Diseases

114. Behçet's disease in Iraqi patients. Full Text available with Trip Pro

, mainly the knees and ankles. The pathergy test was positive in 37 of 52 patients with BD, and negative in all 120 healthy controls and in 20 healthy volunteers who possessed the HLA-B51 antigen. HLA-B51 was present in 32 of 52 (62%) patients with BD compared with 51 of 175 (29%) unrelated normal controls. Both the pathergy and HLA-B51 tests were negative in four of 52 patients with BD. Behçet's disease is not uncommon in Iraq. Practising physicians, dermatologists, and ophthalmologists must be more

1986 Annals of the Rheumatic Diseases

115. Prevalence of Behçet's disease in an Arab community in Israel Full Text available with Trip Pro

lasting more than seven days) during the previous year were invited for an extensive interview and examination by a rheumatologist or a paediatrician.A total of 4876 subjects were included in this survey, of whom six (one male, five female) were diagnosed as having BD. Of these six, two were siblings (a brother and a sister). Five had skin lesions, four had visual involvement, and all had genital ulcers and joint symptoms; one in two patients had a positive pathergy test. Five of the six carried HLA

2002 Annals of the Rheumatic Diseases

116. Behçet's disease associated with amyloidosis in Turkey and in the world. Full Text available with Trip Pro

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

1988 Annals of the Rheumatic Diseases

117. A randomized, controlled and blinded study of papulopustular lesions in Turkish Behçet's patients. (Abstract)

% and the most common location was the trunk, whereas in the control group the frequency was 89% and the most common location was the face. In acne and non-acne patients, the frequency was 100% and 86.1% respectively. The total mean number, and mean numbers of PPL on the location of trunk, upper and lower extremities, and genitalia were higher in patients with BD than in controls. When the PPL in BD patients with a positive pathergy test was compared with that in patients with a negative pathergy test

1998 International journal of dermatology

118. Dapsone in Behçet's disease: a double-blind, placebo-controlled, cross-over study. (Abstract)

100 mg daily or placebo for three months in a double-blind manner. After three months, patients were crossed over and followed for a further three months. Patients were followed up in each visit by assessing the number, size, duration and frequency of oral and genital ulcers, other cutaneous manifestations, and systemic manifestations of the disease. A pathergy test was done on each visit. Laboratory investigations included hemoglobin concentration, white blood cell count, ESR, and C-reactive (...) protein. In dapsone-treated patients, there were significant reductions in the oral and genital ulcer parameters as well as the incidence of other cutanous and systemic manifestations. In the placebo-treated group, there were no significant changes in these parameters. The pathergy test result as well as those of other laboratory tests were all decreased in the dapsone-treated group. Although this study was a small scale study, it shows that dapsone was effective in treatment of mucocutaneous

2002 The Journal of dermatology Controlled trial quality: uncertain

119. Clinical features of patients with Behçet's disease and epididymitis. (Abstract)

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

120. Pathergy Full Text available with Trip Pro

Pathergy 17452660 2007 05 10 2018 11 13 1488-2329 176 9 2007 Apr 24 CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne CMAJ Pathergy. 1275-6 Madan Vishal V The Dermatology Centre, Hope Hospital, Salford, United Kingdom. Lear John J eng Case Reports Journal Article Canada CMAJ 9711805 0820-3946 AIM IM Adult Behcet Syndrome diagnosis Diagnosis, Differential Erythema diagnosis Humans Male Skin Diseases, Vesiculobullous diagnosis 2007 4 25 9 0 2007 5 11 9 0

2007 CMAJ : Canadian Medical Association Journal

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