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Pemphigus

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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

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

4. 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

5. Rituximab (MabThera) Treatment of patients with moderate to severe pemphigus vulgaris.

Rituximab (MabThera) Treatment of patients with moderate to severe pemphigus vulgaris. Published 13 May 2019 Statement of advice SMC2193 rituximab 100mg Concentrate for Solution for Infusion (MabThera®) Roche Products Ltd 5 April 2019 ADVICE: in the absence of a submission from the holder of the marketing authorisation rituximab (MabThera®) is not recommended for use within NHSScotland. Indication under review: treatment of patients with moderate to severe pemphigus vulgaris. The holder

2019 Scottish Medicines Consortium

6. Validation of the BIOCHIP test for the diagnosis of bullous pemphigoid, pemphigus vulgaris and pemphigus foliaceous. (PubMed)

Validation of the BIOCHIP test for the diagnosis of bullous pemphigoid, pemphigus vulgaris and pemphigus foliaceous. The BIOCHIP is a novel multiplex indirect immunofluorescence technique used in the serological diagnosis of bullous pemphigoid and pemphigus. The BIOCHIP method combines the screening of autoantibodies and target antigen specific substrates in a single miniature incubation field.To evaluate the diagnostic accuracy of the new immunofluorescence BIOCHIP multiplex tool in pemphigus

2019 Journal of the European Academy of Dermatology and Venereology

7. 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

8. Evaluation of prolactin levels in patients with newly diagnosed pemphigus vulgaris and its correlation with pemphigus disease area index (PubMed)

Evaluation of prolactin levels in patients with newly diagnosed pemphigus vulgaris and its correlation with pemphigus disease area index Prolactin is a hormone; in addition to it known roles, it has immunomodulatory effects on lymphocytes maturation and immunoglobulins production. Hyperprolactinemia has been demonstrated in various autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis, type I diabetes mellitus, and Graves' disease. In view of the prolactin (...) immunomodulatory roles, studying prolactin levels in pemphigus as an autoimmune blistering disease may introduce new ways of understanding disease etiology and developing treatment strategies.Our purpose was to determine the prolactin levels in patients with newly diagnosed pemphigus vulgaris and study its correlation with pemphigus disease area index.Our study was limited by the lack of a control group.In this cross-sectional study, prolactin and anti-desmoglein 1 and 3 autoantibodies levels were measured

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2016 International journal of women's dermatology

9. British Association of Dermatologists guidelines for the management of pemphigus vulgaris

British Association of Dermatologists guidelines for the management of pemphigus vulgaris GUIDELINES BJD British Journal of Dermatology British Association of Dermatologists’ guidelines for the management of pemphigus vulgaris 2017* K.E. Harman, 1 D. Brown, 2 L.S. Exton, 3 R.W. Groves, 4 P.J. Hampton, 5 M.F. Mohd Mustapa, 3 J.F. Setter?eld 4,6 and P.D. Yesudian 7 1 University Hospitals Leicester, Leicester Royal In?rmary, In?rmary Square, Leicester, LE1 5WW, U.K. 2 St John’s Institute (...) of pemphigus vulgaris (PV). The document aims to update and expand on the previous guidelines by (i) offering an appraisal of all relevant literature from January 2000 up to May 2016, focusing on any key developments; (ii) addressing important, practical, clinical questions relating to the primary guideline objective; (iii) providing guideline recommendations with, where appropriate, some health economic implications and (iv) discussing potential developments and future directions. The guideline

2017 British Association of Dermatologists

10. 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

11. 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

12. 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

13. 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

14. Increased risk of severe course of pemphigus in patients with pemphigus-associated alopecia: a prospective observational study. (PubMed)

Increased risk of severe course of pemphigus in patients with pemphigus-associated alopecia: a prospective observational study. Pemphigus-associated alopecia is considered rare, and has not been studied in detail.To evaluate the clinical and immunological characteristics of patients with pemphigus-associated alopecia.This prospective observational study included 80 consecutive patients with histopathologically and immunopathologically confirmed pemphigus, of whom 11 (13.8%) were found to have (...) pemphigus-associated alopecia. Alopecia was observed in 11/52 patients with pemphigus and scalp involvement: [0/28 (35.7%) with pemphigus vulgaris and 1/24 (4.2%) with pemphigus foliaceus. The clinical and immunological characteristics of these patients were analysed.Patients with pemphigus-associated alopecia had a significantly higher Pemphigus Disease Area Index total activity score compared with patients who had no pemphigus-associated alopecia (21.8 ± 18.6 and 11.0 ± 20.5, respectively; P = 0.02

2018 Clinical & Experimental Dermatology

15. Pemphigus. (PubMed)

Pemphigus. Pemphigus consists of a group of rare and severe autoimmune blistering diseases mediated by pathogenic autoantibodies mainly directed against two desmosomal adhesion proteins, desmoglein (Dsg)1 and Dsg3 (also known as DG1 and DG3), which are present in the skin and surface-close mucosae. The binding of autoantibodies to Dsg proteins induces a separation of neighbouring keratinocytes, in a process known as acantholysis. The two main pemphigus variants are pemphigus vulgaris, which (...) often originates with painful oral erosions, and pemphigus foliaceus, which is characterised by exclusive skin lesions. Pemphigus is diagnosed on the basis of either IgG or complement component 3 deposits (or both) at the keratinocyte cell membrane, detected by direct immunofluorescence microscopy of a perilesional biopsy, with serum anti-Dsg1 or anti-Dsg3 antibodies (or both) detected by ELISA. Corticosteroids are the therapeutic mainstay, which have recently been complemented by the anti-CD20

2019 Lancet

16. 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

17. 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

18. Reviewing putative industrial triggering in pemphigus: cluster of pemphigus in the area near the wastewater treatment plant (PubMed)

Reviewing putative industrial triggering in pemphigus: cluster of pemphigus in the area near the wastewater treatment plant A range of pemphigus is relatively rare potentially fatal group of autoimmune blistering dermatoses. Usually, there is no apparent triggering, while in some predisposed patients there are alleged environmental/industrial inducing factors. In a short time period (4 years), we diagnosed 3 novel cases of pemphigus (1 pemphigus vulgaris, 1 pemphigus foliaceus and 1 shift from (...) pemphigus foliaceus into pemphigus vulgaris) at a clinical and laboratory level (ELISA, immunofluorescence studies). We discuss a possible common inducing mechanism as these patients inhabit one estate of the Poznan suburbia (Kozieglowy, population < 12,000), Greater Poland district, Poland, and review literature data on alleged pemphigus triggers. To the best of our knowledge, this is the first report exploring the putative association between pemphigus diseases and wastewater treatment plant

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2017 Advances in Dermatology and Allergology/Postȩpy Dermatologii i Alergologii

19. 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

20. 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

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