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Pemphigus Foliaceus

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21. Patients affected by endemic pemphigus foliaceus in Colombia, South America exhibit autoantibodies to optic nerve sheath envelope cell junctions Full Text available with Trip Pro

Patients affected by endemic pemphigus foliaceus in Colombia, South America exhibit autoantibodies to optic nerve sheath envelope cell junctions The majority of the patients affected by a new variant of endemic pemphigus foliaceus in El Bagre, Colombia (El Bagre EPF or pemphigus Abreu-Manu), have experienced vision problems; we have previously reported several ocular abnormalities.Here, we aimed to investigate reactivity to optic nerves in these patients. We utilized bovine, rat and mouse optic (...) and the vessels inside it, as well as other molecules inside the nerve. No control cases were positive. Of interest, all the patient autoantibodies co-localized with commercial antibodies to desmoplakins I-II, myocardium-enriched zonula occludens-1- associated protein (MYZAP), armadillo repeat gene deleted in velo-cardio-facial syndrome (ARVCF), and plakophilin-4 (p0071) from Progen Biotechnik (P < 0.001).We conclude that the majority of the patients affected by pemphigus Abreu-Manu have autoantibodies

2018 Dermatology practical & conceptual

22. Validity of the EQ-5D in pemphigus vulgaris and foliaceus patients. Full Text available with Trip Pro

Validity of the EQ-5D in pemphigus vulgaris and foliaceus patients. No studies to date have employed the EuroQoL EQ-5D questionnaire to assess health-related quality of life (HRQoL) in patients with pemphigus.To evaluate the HRQoL of patients with pemphigus by the EQ-5D and to analyse the convergent and known-groups validity of the EQ-5D in this patient population.Between 2014 and 2017, a multicentre cross-sectional study was carried out. Outcome measures included the five-level EQ-5D (EQ-5D-5L (...) with pemphigus vulgaris and those with pemphigus foliaceus (0·81 vs. 0·86, P = 0·14). The mean EQ-5D index scores of patients with limited, moderate, significant and extreme pemphigus were 0·88, 0·82, 0·72 and 0·67, respectively (P = 0·001). The number of comorbidities was associated with greater impairment in EQ-5D index scores (P < 0·001). DLQI (rs = -0·62, P < 0·001) and the average pain intensity VAS (rs = -0·59, P < 0·001) more strongly correlated with the EQ-5D index scores than did ABSIS (rs = -0·40

2018 British Journal of Dermatology

23. Sparking Fire Under the Skin? Answers From the Association of Complement Genes With Pemphigus Foliaceus Full Text available with Trip Pro

Sparking Fire Under the Skin? Answers From the Association of Complement Genes With Pemphigus Foliaceus Skin blisters of pemphigus foliaceus (PF) present concomitant deposition of autoantibodies and components of the complement system (CS), whose gene polymorphisms are associated with susceptibility to different autoimmune diseases. To investigate these in PF, we evaluated 992 single-nucleotide polymorphisms (SNPs) of 44 CS genes, genotyped through microarray hybridization in 229 PF patients

2018 Frontiers in immunology

24. Network meta-analysis-based comparison of first-line steroid-sparing adjuvants in treatment of pemphigus vulgaris and pemphigus foliaceus: a network meta-analysis

Network meta-analysis-based comparison of first-line steroid-sparing adjuvants in treatment of pemphigus vulgaris and pemphigus foliaceus: a network meta-analysis Print | PDF PROSPERO This information has been provided by the named contact for this review. CRD has accepted this information in good faith and registered the review in PROSPERO. CRD bears no responsibility or liability for the content of this registration record, any associated files or external websites. Email salutation (e.g. "Dr

2018 PROSPERO

25. Insights into the epidemiological link between biting flies and pemphigus foliaceus in southeastern Brazil. Full Text available with Trip Pro

Insights into the epidemiological link between biting flies and pemphigus foliaceus in southeastern Brazil. Black fly and sandfly bites are related to the endemicity of pemphigus foliaceus (PF); however, an immune reaction against the salivary proteins from these flies still requires confirmation in the case of PF patients living in southeastern Brazil.To georeference the distribution of Simuliidae (Diptera: Simuliidae) and Phlebotominae (Diptera: Psychodidae) and of PF cases

2017 Acta Tropica

26. HLA class II alleles of susceptibility and protection in Brazilian and Dutch pemphigus foliaceus. (Abstract)

HLA class II alleles of susceptibility and protection in Brazilian and Dutch pemphigus foliaceus. 28977698 2019 02 19 1365-2133 178 3 2018 03 The British journal of dermatology Br. J. Dermatol. HLA class II alleles of susceptibility and protection in Brazilian and Dutch pemphigus foliaceus. e212-e214 10.1111/bjd.16022 de Sena Nogueira Maehara L L 0000-0002-4113-1379 Department of Dermatology, Federal University of São Paulo, São Paulo, Brazil. Center for Blistering Diseases, Department

2017 British Journal of Dermatology

27. Patients with a new variant of endemic pemphigus foliaceus have autoantibodies against arrector pili muscle, colocalizing with MYZAP, p0071, desmoplakins 1 and 2 and ARVCF. (Abstract)

Patients with a new variant of endemic pemphigus foliaceus have autoantibodies against arrector pili muscle, colocalizing with MYZAP, p0071, desmoplakins 1 and 2 and ARVCF. We identified a new variant of endemic pemphigus foliaceus in El Bagre, Colombia, South America, which we term El Bagre-EPF, and observed reactivity to arrector pili muscle (APM), thus we tested for autoimmunity to APM.We took skin biopsies from 30 patients with El Bagre-EPF and 30 healthy controls (HCs) matched by age, sex

2017 Clinical & Experimental Dermatology

28. Cell junction protein armadillo repeat gene deleted in velo-cardio-facial syndrome is expressed in the skin and colocalizes with autoantibodies of patients affected by a new variant of endemic pemphigus foliaceus in Colombia Full Text available with Trip Pro

Cell junction protein armadillo repeat gene deleted in velo-cardio-facial syndrome is expressed in the skin and colocalizes with autoantibodies of patients affected by a new variant of endemic pemphigus foliaceus in Colombia We previously described a new variant of endemic pemphigus foliaceus in El Bagre, Colombia, South America (El Bagre-EPF, or pemphigus Abreu-Manu). El Bagre-EPF differs from other types of EPF clinically, epidemiologically, immunologically and in its target antigens. We

2017 Dermatology practical & conceptual

29. Refractory pemphigus foliaceus associated with herpesvirus infection: case report Full Text available with Trip Pro

Refractory pemphigus foliaceus associated with herpesvirus infection: case report Pemphigus foliaceus (PF) is an autoimmune disease characterized by blistering of the skin. Infections caused by members of the herpesviridae family have been suggested as a possible triggering factor for pemphigus vulgaris (PV), but not for PF. The present study aimed to investigate the presence of Human herpesvirus (types 1, 2, 3) in corticosteroid refractory skin lesions from a patient with PF, by a Polymerase

2017 Revista do Instituto de Medicina Tropical de São Paulo

30. Cytokine and Chemokines Alterations in the Endemic Form of Pemphigus Foliaceus (Fogo Selvagem) Full Text available with Trip Pro

Cytokine and Chemokines Alterations in the Endemic Form of Pemphigus Foliaceus (Fogo Selvagem) The endemic form (fogo selvagem-FS) of pemphigus foliaceus is an autoimmune disease characterized by the presence of IgG autoantibodies against desmoglein-1. Despite the array of findings, the role of chemokines and cytokines that dictate the immune response and disease outcome is still poorly investigated.Serum from 64 patients diagnosed with FS was used to draw and establish the levels

2017 Frontiers in immunology

31. Divergent Specificity Development of IgG1 and IgG4 Autoantibodies in Endemic Pemphigus Foliaceus (Fogo Selvagem) Full Text available with Trip Pro

Divergent Specificity Development of IgG1 and IgG4 Autoantibodies in Endemic Pemphigus Foliaceus (Fogo Selvagem) We have shown that although the IgG response in fogo selvagem (FS) is mainly restricted to desmoglein (Dsg) 1, other keratinocyte cadherins are also targeted by FS patients and healthy control subjects living in the endemic region of Limão Verde, Brazil (endemic controls). Evaluating nonpathogenic IgG1 and pathogenic IgG4 subclass responses to desmosomal proteins may reveal important

2017 ImmunoHorizons

32. Putative contact ketoconazole shampoo-triggered pemphigus foliaceus in a dog Full Text available with Trip Pro

Putative contact ketoconazole shampoo-triggered pemphigus foliaceus in a dog A 10-year-old spayed female cocker spaniel dog was referred for an evaluation of acute-onset generalized pustular cutaneous lesions following application of ketoconazole shampoo. Cytologic and histopathologic examinations of the lesions revealed intra-epidermal pustules with predominantly neutrophils and acantholytic cells. This is the first description of putative contact ketoconazole shampoo-triggered pemphigus (...) foliaceus in a dog.

2017 The Canadian Veterinary Journal

33. The Use of Intralesional Steroids in a Case of Localized Pemphigus Foliaceus Full Text available with Trip Pro

The Use of Intralesional Steroids in a Case of Localized Pemphigus Foliaceus A 37-year-old South-Asian male presented to our clinic with a crusty, verrucous-like, scaly plaque of the left ala of the nose. After ruling out infectious and other epidermal bullous diseases, we finalized a diagnosis of localized pemphigus foliaceus, an exceptionally rare disorder with only 15 cases reported in the literature to date. The hyperkeratotic lesions responded favorably to a 3-week regimen of triamcinolone

2017 Case reports in dermatology

34. A Safety and Dose-Finding Study of SYNT001 in Subjects With Pemphigus (Vulgaris or Foliaceus)

A Safety and Dose-Finding Study of SYNT001 in Subjects With Pemphigus (Vulgaris or Foliaceus) A Safety and Dose-Finding Study of SYNT001 in Subjects With Pemphigus (Vulgaris or Foliaceus) - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one (...) or more studies before adding more. A Safety and Dose-Finding Study of SYNT001 in Subjects With Pemphigus (Vulgaris or Foliaceus) The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. of clinical studies and talk to your health care provider before participating. Read our for details. ClinicalTrials.gov Identifier: NCT03075904 Recruitment Status : Recruiting

2017 Clinical Trials

35. Pemphigus Foliaceus

Pemphigus Foliaceus Pemphigus Foliaceus Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Pemphigus Foliaceus Pemphigus Foliaceus Aka (...) : Pemphigus Foliaceus , Superficial Pemphigus , Pemphigus erythematosus , Senear-Usher Syndrome , Fogo selvagem , Pemphigus herpetiformis From Related Chapters II. Definition Less common, milder and superficial form of III. Signs Onset on skin of face in butterfly distribution May also involve scalp, chest or upper back Rarely involves absent IV. Variants Pemphigus erythematosus (Senear-Usher Syndrome) Localized variant of Pemphigus Foliaceus Associated with positive Fogo selvagem Variant of Pemphigus

2018 FP Notebook

36. Pemphigus

Pemphigus Pemphigus - Symptoms, diagnosis and treatment | BMJ Best Practice You'll need a subscription to access all of BMJ Best Practice Search  Pemphigus Last reviewed: February 2019 Last updated: January 2019 Summary Group of autoimmune blistering diseases. Pemphigus foliaceus (PF) is confined to the skin. Pemphigus vulgaris (PV) and paraneoplastic pemphigus (PNP) can involve the skin and mucosal surfaces of the eyes, mouth, nasopharynx, and oesophagus. Diagnosis is based on clinical (...) non-Hodgkin's lymphoma, and mortality can approach 90% due to respiratory failure from bronchiolitis obliterans. Definition Pemphigus describes a group of autoimmune blistering diseases that involve the epidermal surfaces of the skin, mucosa, or both. There are 3 broad categories: pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP). Pemphigus is mediated by an autoantibody that binds a specific component of the desmosomal plaque. The desmosomal plaque holds

2019 BMJ Best Practice

37. Differential HLA class I and class II associations in pemphigus foliaceus and pemphigus vulgaris patients from a prevalent Southeastern Brazilian region. (Abstract)

Differential HLA class I and class II associations in pemphigus foliaceus and pemphigus vulgaris patients from a prevalent Southeastern Brazilian region. Genetic factors, particularly those concerning HLA class II, have been associated with the pathogenesis of pemphigus. Taking advantage of an area where pemphigus foliaceus (PF) and pemphigus vulgaris (PV) are prevalent in the northeastern region of the state of São Paulo, Southeastern Brazil, we have studied the HLA class I (A, B and C (...) and the HLA-DRB1*01-DQA1*01-DQB1*05 haplotype in PF patients and the HLA-DRB1*04:02 and *14:01 alleles and the HLA-DRB1*14-DQA1*01-DQB1*05 haplotype in PV patients were related with the highest etiologic fraction values. Distinct genetic patterns and not yet described HLA susceptibility/protection alleles/haplotypes profiles have been observed in this series. Our findings corroborate the differential genetic markers in PF and PV in an area where pemphigus is prevalent but not yet reported.Copyright © 2016

2016 Journal of Autoimmunity

38. Pemphigus

Pemphigus Pemphigus - Symptoms, diagnosis and treatment | BMJ Best Practice You'll need a subscription to access all of BMJ Best Practice Search  Pemphigus Last reviewed: February 2019 Last updated: January 2019 Summary Group of autoimmune blistering diseases. Pemphigus foliaceus (PF) is confined to the skin. Pemphigus vulgaris (PV) and paraneoplastic pemphigus (PNP) can involve the skin and mucosal surfaces of the eyes, mouth, nasopharynx, and oesophagus. Diagnosis is based on clinical (...) non-Hodgkin's lymphoma, and mortality can approach 90% due to respiratory failure from bronchiolitis obliterans. Definition Pemphigus describes a group of autoimmune blistering diseases that involve the epidermal surfaces of the skin, mucosa, or both. There are 3 broad categories: pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP). Pemphigus is mediated by an autoantibody that binds a specific component of the desmosomal plaque. The desmosomal plaque holds

2018 BMJ Best Practice

39. Prediction of survival for patients with pemphigus vulgaris and pemphigus foliaceus: a retrospective cohort study Full Text available with Trip Pro

Prediction of survival for patients with pemphigus vulgaris and pemphigus foliaceus: a retrospective cohort study Factors associated with survival in pemphigus have not yet been thoroughly addressed. Therefore, in the present study, risk factors for overall mortality in a large group of patients with pemphigus vulgaris and foliaceus were investigated.A retrospective hospital-based cohort study was carried out, between October 1998 and November 2012, in the Department of Dermatology (...) of the University of Medicine and Pharmacy "Iuliu Hatieganu", Cluj-Napoca, Romania. The investigated prognostic endpoint was the overall survival of the patients.A total of 130 patients were studied (108 with pemphigus vulgaris and 22 with pemphigus foliaceus). In pemphigus vulgaris group, univariate analysis found a statistically significant association between the age of onset ≥ 65 years (p < 0.001), presence of coronary heart disease (p = 0.006), presence of cardiac arrhythmia (p = 0.004), level of anti

2015 Orphanet journal of rare diseases

40. Valuation of pemphigus vulgaris and foliaceus health states: a convenience sample experiment. (Abstract)

Valuation of pemphigus vulgaris and foliaceus health states: a convenience sample experiment. Health-related quality of life (HRQoL) in pemphigus has been widely investigated; nevertheless, utility values for economic evaluations are still lacking.To estimate health utilities for hypothetical pemphigus vulgaris (PV) and pemphigus foliaceus (PF) health states in a general population sample.Three health states (uncontrolled PV, uncontrolled PF and controlled pemphigus) were developed based (...) on a systematic literature review of HRQoL studies in pemphigus. Utilities were obtained from a convenience sample of 108 adults using a visual analogue scale (VAS) and 10-year time trade-off (TTO). Lead-time TTO was applied for health states regarded as worse than dead with a lead time to disease time ratio of 1 : 1.The mean VAS utility scores for PV, PF and controlled pemphigus were 0·25 ± 0·15, 0·37 ± 0·17 and 0·63 ± 0·16, respectively. Corresponding TTO utilities were as follows: 0·34 ± 0·38, 0·51 ± 0·32

2016 British Journal of Dermatology

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