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Pemphigus Foliaceus

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1. Pemphigus Foliaceus and Pemphigus Erythematosus are the Most Common Subtypes of Pemphigus in Northern Finland. (PubMed)

Pemphigus Foliaceus and Pemphigus Erythematosus are the Most Common Subtypes of Pemphigus in Northern Finland. Pemphigus is an autoimmune skin disease characterized by blistering and erosions of the skin and mucous membranes. Pemphigus is rare in Northern and Western Europe but its incidence is higher around the Mediterranean Sea. The most common type worldwide is pemphigus vulgaris. The aim of this study was to investigate the incidence of pemphigus subtypes in Northern Finland between 1985 (...) and 2017. A total of 46 patients diagnosed with pemphigus at the Department of Dermatology of Oulu University Hospital were found the female/male ratio was 1.7. In contrast to many other countries it was found that in Northern Finland the superficial pemphigus subtypes were the most common: erythematosus or foliaceus (65%) followed by pemphigus vulgaris (26%). Over the past 4 decades the annual incidence of pemphigus in Finland has increased from 0.76 to 2.8 cases per million persons.

2019 Acta Dermato-Venereologica

2. Pemphigus vulgaris and pemphigus foliaceus: an overview of the clinical presentation, investigations and management. (PubMed)

Pemphigus vulgaris and pemphigus foliaceus: an overview of the clinical presentation, investigations and management. Pemphigus diseases are cutaneous and mucous membrane blistering diseases, related to the key antigens of desmoglein 1 and 3. This article reviews the topic, including diagnosis, and provides the physician with guidance on the treatment of these difficult to control disorders.© 2019 British Association of Dermatologists.

2019 Clinical & Experimental Dermatology

3. New biochip immunofluorescence test for the serological diagnosis of pemphigus vulgaris and foliaceus: A review of the literature (PubMed)

New biochip immunofluorescence test for the serological diagnosis of pemphigus vulgaris and foliaceus: A review of the literature The immunoassays that are available for the serological diagnosis of the more common subtypes of autoimmune blistering diseases such as pemphigus vulgaris (PV) and pemphigus foliaceus (PF) include enzyme-linked immunosorbent assay (ELISA) testing to specific antigens desmoglein (Dsg)1 and Dsg3, direct immunofluorescence (DIF), indirect immunofluorescence (IIF (...) ), and immunoblotting. A review of the literature on the biochip assay was conducted. Six studies investigated the validity of a new biochip, mosaic-based, IIF test in patients with pemphigus and demonstrated its relatively high sensitivity and specificity (Dsg3: 97.62-100%, 99.6-100%; Dsg1: 90%, 100%) in comparison with ELISA (Dsg3: 81-100%, 94-100%; Dsg1: 69-100%, 61.1-100%), and/or IIF (PV: 75-100%, 91.8-100%; PF: 67-100%) using suitable substrates. So far, validation studies of the biochip have been conducted

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2018 International journal of women's dermatology

4. Incidence of pemphigus vulgaris and pemphigus foliaceus in North-East Poland (Podlaskie Province) - a 15-year (2001-2015) bicentric retrospective study. (PubMed)

Incidence of pemphigus vulgaris and pemphigus foliaceus in North-East Poland (Podlaskie Province) - a 15-year (2001-2015) bicentric retrospective study. The average incidence of pemphigus reported from European countries ranged between 0.5 and eight cases per one million inhabitants. Studies on epidemiology of pemphigus group in entire Province of Poland over a period of more than a decade are lacking.To analyze the incidence of pemphigus vulgaris (PV) and pemphigus foliaceus (PF) in Podlaskie (...) Province (North-East Poland) in years 2001-2015 with regard to patients' age, gender, and residency.Sixty-six cases of pemphigus were diagnosed in the period analyzed: 44 cases of PV (66.67%) and 22 of PF (33.33%). Female to male ratio was 2.88. The average incidence of PV and PF together was 3.67 ± 2.19 per one million inhabitants. In females the incidence was 5.34 ± 3.20 and in males, 1.90 ± 1.91 (P < 0.05). The incidence in inhabitants of rural areas was 4.96 ± 4.07 and those of urban areas, 2.70

2018 International Journal of Dermatology

5. Exploring the in situ expression of vascular endothelial growth factor and endoglin in pemphigus foliaceus variants and pemphigus vulgaris. (PubMed)

Exploring the in situ expression of vascular endothelial growth factor and endoglin in pemphigus foliaceus variants and pemphigus vulgaris. Erythroderma is a severe manifestation of pemphigus foliaceus (PF), a blistering disease mediated by IgG autoantibodies against desmoglein 1. Increasing evidence supports the contribution of angiogenic mediators in the pathogenesis of erythroderma.To evaluate the in situ expression of vascular endothelial growth factor (VEGF) and endoglin in patients (...) with PF with erythroderma.Formalin-fixed paraffin-embedded skin samples obtained from patients with erythrodermic PF (n = 19; 12 patients with endemic PF), non-erythrodermic PF (n = 17), pemphigus vulgaris (PV; n = 10), psoriasis (n = 10) and healthy individuals (HI; n = 10) were processed in an automated immunohistochemistry platform utilizing anti-VEGF and anti-endoglin as primary antibodies. Reactivity was evaluated both manually (0 = negative; 1+ = mild; 2+ = intense) and through an automated

2018 Journal of the European Academy of Dermatology and Venereology

6. A Case of Concomitant Pemphigus Foliaceus and Oral Pemphigus Vulgaris (PubMed)

A Case of Concomitant Pemphigus Foliaceus and Oral Pemphigus Vulgaris Pemphigus is a chronic autoimmune condition that can affect multiple areas of the body. The two main subtypes of pemphigus are pemphigus vulgaris (PV) and pemphigus foliaceus (PF) which can rarely occur concurrently or even transition from one to the other. The process of transition may be explained by qualitative changes in desmoglein autoantibody profile. We present a rare case of concomitant PF and oral PV and explore (...) the literature on transitions between pemphigus subtypes and whether this case could represent a transition from PF to PV. Furthermore, the realities of multidisciplinary patient management are discussed.

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2018 Head and neck pathology

7. Pemphigus herpetiformis in South Tunisia: a clinical expression of pemphigus foliaceus? (PubMed)

Pemphigus herpetiformis in South Tunisia: a clinical expression of pemphigus foliaceus? Pemphigus herpetiformis (PH) is a rare subtype of pemphigus combining clinical features of dermatitis herpetiformis and the immunopathologic characteristics of pemphigus. We aimed to analyze the epidemiological, clinical, and immunological presentation and management of the disease in a cohort of south Tunisian patients with a long-term follow-up.We included all patients with confirmed PH followed from (...) % of cases, respectively.PH is characterized by the occurrence of exclusive skin lesions in young women with a good response to corticosteroids but not to Dapsone. Reactivity mainly against Dsg1 suggests that PH in south Tunisia is a particular variant of nonendemic pemphigus foliaceus.© 2018 The International Society of Dermatology.

2018 International Journal of Dermatology

8. New onset pemphigus foliaceus during pregnancy: A rare case (PubMed)

New onset pemphigus foliaceus during pregnancy: A rare case Pemphigus is a rare autoimmune disease during pregnancy. Among the different pemphigus types, pemphigus foliaceus (PF) is even rarer than pemphigus vulgaris. We present a case of PF in a 36-year-old female patient who presented with PF onset during pregnancy in the form of a disseminated, erythematosquamous rash. A diagnosis was made on the basis of histologic, immunofluorescence, and enzyme-linked immunosorbent assay results (...) . A complete remission was recorded a month after steroid treatment initiation. The patient delivered a premature (33 weeks) but otherwise healthy baby girl. Only three cases of PF have been reported in two retrospective studies found in the English-language bibliography. Although pemphigus during pregnancy is a rare disease and treatment guidelines have not yet been elucidated, the management of these cases is individually evaluated. In all cases, the primary goal should be the control of the maternal

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2017 International journal of women's dermatology

9. Long non-coding RNA polymorphisms influence susceptibility to endemic pemphigus foliaceus. (PubMed)

Long non-coding RNA polymorphisms influence susceptibility to endemic pemphigus foliaceus. Pemphigus foliaceus (PF) is an epidermal autoimmune disease, characterized by the presence of autoantibodies against the desmosomal protein desmoglein 1. Genetic and environmental factors contribute to PF, a complex disease that is endemic in Brazil and Colombia and neighbouring countries, and in Tunisia. Long non-coding RNAs (lncRNAs) may participate in gene regulation by interacting with DNA, proteins (...) microRNA-lncRNA interactions.We showed, for the first time, that variation in lncRNA genes may influence pemphigus pathogenesis. Our findings highlight the importance of lncRNA variation in autoimmune and possibly other complex diseases and suggest polymorphisms for functional validation. This article is protected by copyright. All rights reserved.This article is protected by copyright. All rights reserved.

2019 British Journal of Dermatology

10. Patient-assigned health utility values for controlled and uncontrolled pemphigus vulgaris and foliaceus. (PubMed)

Patient-assigned health utility values for controlled and uncontrolled pemphigus vulgaris and foliaceus. The assessment of health-related quality of life (HRQoL) in patients with pemphigus is now of increasing interest due to the availability of highly effective new therapies. Preference-based HRQoL values or health utilities required for medical and financial decision-making are not yet available directly from pemphigus patients.To obtain health utility values for current health (...) and hypothetical health states from the perspective of pemphigus patients.A cross-sectional questionnaire survey was carried out with pemphigus patients. Disease severity was rated by Autoimmune Bullous Skin Disorder Intensity Score (ABSIS). Patients were asked to evaluate their current health as well as three common hypothetical pemphigus health states [uncontrolled pemphigus vulgaris (PV), uncontrolled pemphigus foliaceus (PF) and controlled PV/PF] by using composite time trade-off (cTTO). Multiple

2019 Journal of the European Academy of Dermatology and Venereology

11. Herpes simplex virus 1 and cytomegalovirus are associated with pemphigus vulgaris but not with pemphigus foliaceus disease. (PubMed)

Herpes simplex virus 1 and cytomegalovirus are associated with pemphigus vulgaris but not with pemphigus foliaceus disease. Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are blistering autoimmune diseases that depend on interaction between genetic and environmental factors. Viral infections, like herpes simplex viruses 1 and 2 (HSV1/2), cytomegalovirus (CMV), Epstein-Barr virus and dengue virus, could trigger or exacerbate pemphigus. IgM and IgG antibodies against these viruses in serum

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2017 Experimental Dermatology

12. Pemphigus Vulgaris and Pemphigus Foliaceus: Differences in Epidemiology and Mortality. (PubMed)

Pemphigus Vulgaris and Pemphigus Foliaceus: Differences in Epidemiology and Mortality. Little is known about differences in epidemiological features and prognosis between pemphigus vulgaris (PV) and pemphigus foliaceus (PF). The objective of this study was to compare PV and PF patients regarding ethnic variations and mortality rates. Mortality of PV and PF patients was compared with age- and sex-matched control subjects in the general population. The study cohort comprised 207 patients with PV

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2017 Acta Dermato-Venereologica

13. Epidemiologic and genetic data suggest the presence of an endemic form of pemphigus foliaceus in Gran Canaria, Spain. (PubMed)

Epidemiologic and genetic data suggest the presence of an endemic form of pemphigus foliaceus in Gran Canaria, Spain. 30362104 2019 01 01 1365-2133 2018 Oct 25 The British journal of dermatology Br. J. Dermatol. Epidemiological and genetic data suggest the presence of an endemic form of pemphigus foliaceus in Gran Canaria, Spain. 10.1111/bjd.17346 Grau-Pérez M M https://orcid.org/0000-0002-7628-3495 Department of Dermatology, Hospital Universitario de Gran Canaria Dr Negrin, Las Palmas de Gran

2018 British Journal of Dermatology

14. A rare case with prominent features of both discoid lupus erythematosus and pemphigus foliaceus. (PubMed)

A rare case with prominent features of both discoid lupus erythematosus and pemphigus foliaceus. 29858874 2019 01 18 1468-3083 33 1 2019 Jan Journal of the European Academy of Dermatology and Venereology : JEADV J Eur Acad Dermatol Venereol A rare case with prominent features of both discoid lupus erythematosus and pemphigus foliaceus. e5-e7 10.1111/jdv.15099 Bilgic Temel A A Dermatology Department, UNSW, St. George Hospital, Sydney, NSW, Australia. Ergün E E Dermatology and Venereology

2018 Journal of the European Academy of Dermatology and Venereology

15. Validity of the EQ-5D in pemphigus vulgaris and foliaceus patients. (PubMed)

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

16. Pathogenic IgG4 autoantibodies from endemic pemphigus foliaceus recognize a desmoglein-1 conformational epitope. (PubMed)

Pathogenic IgG4 autoantibodies from endemic pemphigus foliaceus recognize a desmoglein-1 conformational epitope. Fogo Selvagem (FS), the endemic form of pemphigus foliaceus, is mediated by pathogenic IgG4 autoantibodies against the amino-terminal extracellular cadherin domain of the desmosomal cadherin desmoglein 1 (Dsg1). Here we define the detailed epitopes of these pathogenic antibodies. Proteolytic footprinting showed that IgG4 from 95% of FS donor sera (19/20) recognized a 16-residue

2018 Journal of Autoimmunity

17. Sparking Fire Under the Skin? Answers From the Association of Complement Genes With Pemphigus Foliaceus (PubMed)

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

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2018 Frontiers in immunology

18. Patients affected by endemic pemphigus foliaceus in Colombia, South America exhibit autoantibodies to optic nerve sheath envelope cell junctions (PubMed)

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

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2018 Dermatology practical & conceptual

19. Involvement of the IL23/Th17 Pathway in the Pathogenesis of Tunisian Pemphigus Foliaceus (PubMed)

Involvement of the IL23/Th17 Pathway in the Pathogenesis of Tunisian Pemphigus Foliaceus Pemphigus foliaceus (PF) is a rare autoimmune skin disease caused by anti-Dsg1 pathogenic autoantibodies. It is considered as a Th2-mediated disease. Likewise, Th17 cells were recently described in the pathogenesis of the disease but their role is still unclear. We aimed to unravel the eventual implication of the IL23/Th17 pathway in the development of PF. A case-control study was conducted on 115 PF

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2018 Mediators of inflammation

20. Subclinical oral involvement in patients with endemic pemphigus foliaceus (PubMed)

Subclinical oral involvement in patients with endemic pemphigus foliaceus We have described a variant of endemic pemphigus foliaceus (EPF) in El Bagre area known as pemphigus Abreu-Manu. Our previous study suggested that Colombian EPF seemed to react with various plakin family proteins, such as desmoplakins, envoplakin, periplakin BP230, MYZAP, ARVCF, p0071 as well as desmoglein 1.To explore whether patients affected by a new variant of endemic pemphigus foliaceus (El Bagre-EPF) demonstrated (...) in the glands and were positive in 41 of 45 patients and 3 of 45 controls.Patients affected by El Bagre-EPF have some oral anomalies and an immune response, primarily to cell junctions. The intrinsic oral mucosal immune system, including IgA and secretory IgA, play an important role in this autoimmunity. Our data contradict the hypothesis that pemphigus foliaceus does not affect the oral mucosa due to the desmoglein 1-desmoglein 3 compensation.

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2018 Dermatology practical & conceptual

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