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41. 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

2018 FP Notebook

42. Pyoderma gangrenosum during pregnancy - treatment options revisited. (PubMed)

for the health of the mother and the unborn.In pregnant females, it is particularly important to diagnose PG and control disease activity due to the risk of pathergy and wound healing deficiencies during delivery and post-partum. A limited number of treatment options are available to date, which require a precise risk-benefit evaluation.© 2016 European Academy of Dermatology and Venereology.

2016 Journal of the European Academy of Dermatology and Venereology

43. Successful Treatment of Pyoderma Gangrenosum after Augmentation Mastopexy Using Vacuum Therapy (PubMed)

Successful Treatment of Pyoderma Gangrenosum after Augmentation Mastopexy Using Vacuum Therapy Pyoderma gangrenosum (PG) is a rare, severe, destructive neutrophilic dermatosis characterized by a progressive, necrotizing process after skin injury. Its cause is still unknown, and diagnosis represents a challenge when ulcers are seen after surgery. Bacterial infection is not found in the wounds. Patients exhibit "pathergy" with the appearance of new lesions after local trauma such as surgical

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2016 Plastic and Reconstructive Surgery Global Open

44. Severe Bilateral Lower Extremity Pyoderma Gangrenosum (PubMed)

of Surgery, University of South Florida Morsani College of Medicine, Tampa. Payne Wyatt G WG C.W. Bill Young Bay Pines VA Medical Center, Bay Pines, Fla. eng Case Reports 2016 11 21 United States Eplasty 101316107 1937-5719 TNF-α inhibitors lower extremities pathergy pyoderma gangrenosum wound 2016 12 17 6 0 2016 12 17 6 0 2016 12 17 6 1 epublish 27980702 PMC5120372 Wounds. 2009 Apr;21(4):89-94 25903230 Am J Clin Dermatol. 2012 Jun 1;13(3):191-211 22356259 Br J Dermatol. 2015 Jun;172(6):1487-97 25350484

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2016 Eplasty

45. Successful treatment of Behçet’s uveitis with Tocilizumab (PubMed)

Successful treatment of Behçet’s uveitis with Tocilizumab A 33-year-old man with history of lymphoma was misdiagnosed as multiple sclerosis (MS) 18 months previously, developed intermediate uveitis, unresponsive to interferon, and corticosteroids. A diagnosis of Behçet's disease (BD) was made on the basis of recurrent orogenital ulceration, erythema nodosum, and a positive pathergy test. The patient was started on Tocilizumab and experienced an improvement in visual acuity and intraocular

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2016 Saudi Journal of Ophthalmology

46. Investigation of Behçet’s Disease and Recurrent Aphthous Stomatitis Frequency: The Highest Prevalence in Turkey (PubMed)

hospital, and the Pathergy Test and eye examinations were performed.The annual prevalence of RAS was determined as 10.84%. The annual prevalence was determined to be higher in women than in men (p=0.000). It was observed that the prevalence was at the peak level in the 3(rd) decade, and then decreased proportionally in the following decades (p=0.000). It was also observed that the aphtha recurrence decreased in the following decades (p=0.048). The Behçet's prevalence was found to be 0.60

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2016 Balkan medical journal

47. Postoperative Pyoderma Gangrenosum: A Clinical Review of Published Cases. (PubMed)

Postoperative Pyoderma Gangrenosum: A Clinical Review of Published Cases. Postoperative pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by PG at surgical incisions. It is often misdiagnosed as wound infection, and pathergy may complicate wound debridement. From September 1, 2013, through November 30, 2013, a literature search was conducted of articles published from January 1, 1978, through December 31, 2012. We referenced PubMed, MEDLINE, and Mayo Clinic Libraries using

2016 Mayo Clinic Proceedings

49. Use of Becaplermin for nondiabetic ulcers: pyoderma gangrenosum and calciphylaxis (PubMed)

gangrenosum (PG), a neutrophilic dermatosis. We also report that topical collagenase worsened PG ulcers, consistent with pathergy. Becaplermin can be used to help treat ulcers resulting from calciphylaxis and PG. These encouraging results lend support for the utilization of becaplermin in the treatment of nondiabetic chronic ulcers of various etiologies. © 2015 Wiley Periodicals, Inc.

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2015 Dermatologic therapy

50. Ulcus vulvae acutum — A case of genital ulcers in adolescent girl (PubMed)

, inflammatory, traumatic, and neoplastic causes. We describe a case of adolescent female with an episode of ulcus vulvae acutum associated with infectious mononucleosis. The diagnosis was supported by the clinical symptoms, elevated circulating levels of liver enzymes, positive EBV serology, cervical and inguinal lymphadenomegaly, and hepatosplenomegaly. The patient presented a history of aphthous stomatitis. Negative Pathergy test and the absence of any other related symptoms allowed us to exclude

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2015 Case Reports in Women's Health

51. Behçet's disease in black skin. A retrospective study of 50 cases in Dakar. (PubMed)

women. Two patients had a positive family history of Behçet's disease. The oral and genital aphthous lesions were present in 100% of patients. The pathergy test was positive in 16 patients (32%). Following skin conditions were observed: pseudofolliculitis in 15 patients (30%), acneiform papules in 6 patients (12%), erythema nodosum in 4 patients (8%) and leg ulcers in one patient. Ocular involvement was reported in 22 patients (44%) and joint involvement in 20 patients (40%). Neurological

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2015 Journal of dermatological case reports

52. Characterization of the major histocompatibility complex locus association with Behçet’s disease in Iran (PubMed)

correlations do not survive Bonferroni correction, while carriers of the rs76546355 risk allele predominate in BD cases with genital ulcers, positive pathergy test and positive BD family history (2.31 × 10(-4) ≤ P ≤ 1.59 × 10(-3)).We found that the HLA-B*51 allele and the rs76546355/rs116799036 MHC SNP are independent genetic risk factors for BD in Iranian, and that positivity for the rs76546355/rs116799036 risk allele, but not for B*51, does correlate with specific demographic characteristics or clinical

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2015 Arthritis research & therapy

53. Interleukin-10 gene polymorphisms are associated with Behcet’s disease but not with Vogt-Koyanagi-Harada syndrome in the Chinese Han population (PubMed)

with extraocular findings, including genital ulcers, skin lesions, and a positive pathergy test. No difference was found among the mRNA expressions of IL-10 in the peripheral blood mononuclear cells (PBMCs) of controls with different genotypes of rs1800871 after stimulation of lipopolysaccharide (LPS) or anti-CD3/CD28 antibodies.The findings showed that IL-10 is a risk gene for BD but not for VKH syndrome.

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2015 Molecular vision

54. Sweet's syndrome following surgery: Cutaneous trauma as a possible aetiological co-factor in neutrophilic dermatoses. (PubMed)

with the development of Sweet's syndrome and resolution was obtained after the initiation of dapsone. We propose that surgery may produce an acute inflammatory response in a similar manner to pathergy reactions, which play an aetiological role in other neutrophilic dermatoses, such as pyoderma gangrenosum. We conclude that there may be greater overlap between these neutrophilic dermatoses than previously appreciated. © 2014 The Australasian College of Dermatologists.

2014 Australasian Journal of Dermatology

55. An unusual case of bilateral Pyoderma Gangrenosum with Achilles tendon rupture. (PubMed)

after the left ankle developed a foul-smelling discharging wound. Repeat debridement led to worsening of the condition (pathergy). Intraoperative tissue cultures and microscopy showed no evidence of fungi, bacteria or mycobacteria. Histology showed granulation, inflammatory infiltrate, abscess formation and focal necrotizing vasculitis. Dermatology opinion confirmed PG. Awareness of the diagnosis of PG, and early involvement of dermatology, in a rapidly progressing wound is essential to avoid (...) delayed treatment and prevent worsening through pathergy. © 2014 British Association of Dermatologists.

2014 British Journal of Dermatology

56. Gender-specific differences in Adamantiades-Behçet's disease manifestations: an analysis of the German registry and meta-analysis of data from the literature. (PubMed)

with the pathergy test (RR 1.14) and female gender associated with erythema nodosum (RR 0.86). HLA-B5 and Turkish or German origin did not affect the observed associations.These analyses support gender-associated clinical variations in ABD and in particular a clinically meaningful risk of cardiovascular involvement for men.© The Author 2014. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

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2014 Rheumatology (Oxford, England)

57. Pyoderma gangrenosum: a systematic review. (PubMed)

Pyoderma gangrenosum: a systematic review. Pyoderma gangrenosum (PG) is a rare, chronic neutrophilic dermatosis of unknown etiology. The world wide incidence is estimated to be around 3-10 cases per million population per year. In 50-70% of cases inflammatory bowel diseases, hematological malignancies or rheumatologic disorders are associated to PG. Although the etiology is uncertain, the dysregulation of the immune system appears to be implied. Pathergy is the most important triggering factor

2014 Giornale italiano di dermatologia e venereologia : organo ufficiale, Società italiana di dermatologia e sifilografia

58. Behcet Disease (Follow-up)

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

59. 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

60. 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

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