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Pemphigus Foliaceus

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181. Evaluation of coexistence of the Human Herpesvirus type 8 (HHV-8) infection and pemphigus. (PubMed)

of this study is investigation of the association of pemphigus with HHV-8 and the relationship between inflammatory and acantholytic cells with HHV-8 infection.Tissue-extracted DNA from 41 paraffin-embedded skin tissues from patients first presenting with pemphigus was tested using nested PCR for the presence of HHV-8. A total of 37 cases had pemphigus vulgaris (PV) and 4 patients had pemphigus foliaceus (PF). For our control group, normal skin of 21 cosmetic surgery patients were included. Furthermore (...) Evaluation of coexistence of the Human Herpesvirus type 8 (HHV-8) infection and pemphigus. Human Herpesvirus type 8 (HHV-8) is a new member of the herpes virus family, first found in the tissue of acquired immune deficiency syndrome (AIDS)-related Kaposi's sarcoma. Environmental factors including viral infection may play a role in the onset and/or development of pemphigus. Some studies based on polymerase chain reaction findings suggest an association between HHV-8 and pemphigus. The aim

2010 International Journal of Dermatology

182. Immunofluorescence of the outer root sheath: an aid to diagnosis in pemphigus. (PubMed)

samples were also collected from 50 controls.Immunological deposits characteristic of pemphigus were found in the ORS of anagen and telogen hairs in all 50 patients, but in none of the 50 controls. Positive DIF findings were seen in scalp hair irrespective of the presence of scalp lesions. Of the 50 patients with pemphigus, 46 were diagnosed with pemphigus vulgaris, 2 with pemphigus vegetans, and 1 each with pemphigus foliaceus and pemphigus erythematosus, based on the clinical, histopathological (...) Immunofluorescence of the outer root sheath: an aid to diagnosis in pemphigus. The direct immunofluorescence (DIF) pattern of the outer root sheath (ORS) of the hair follicle in patients with pemphigus resembles that of perilesional skin. We performed DIF on anagen and telogen hair ORS from patients with pemphigus and compared the findings with those of perilesional skin.To assess the sensitivity of DIF in the ORS of anagen and telogen hair in patients with pemphigus with positive DIF results

2010 Clinical & Experimental Dermatology

183. Serum plakophilin-3 autoreactivity in paraneoplastic pemphigus. (PubMed)

construct, both encoding human PKP3 (protein products of 85 kDa and 115 kDa, respectively). Protein lysates were made in Laemmli buffer. The proteins were separated by gel electrophoresis, transferred to filters and probed with five PNP sera, four pemphigus vulgaris sera, two pemphigus foliaceus sera, five bullous pemphigoid sera, one cicatricial pemphigoid serum and one linear IgA dermatosis serum. A mouse monoclonal anti-PKP3 antibody raised against a 20-amino acid peptide of human PKP3 was used (...) Serum plakophilin-3 autoreactivity in paraneoplastic pemphigus. Paraneoplastic pemphigus (PNP) is a malignancy-associated autoimmune disease in which circulating autoantibodies recognize various polypeptides that constitute the desmosomes and hemidesmosomes of epithelial structures.To determine whether PNP is associated with autoreactivity against the armadillo-repeat-containing plakophilin-3 (PKP3) protein.HEK293 cells were transiently transfected with either a pEF6/myc-His or a pEGFP-N2

2010 British Journal of Dermatology

184. Antigen Selection of Anti-DSG1 Autoantibodies During and Before the Onset of Endemic Pemphigus Foliaceus. (Full text)

Antigen Selection of Anti-DSG1 Autoantibodies During and Before the Onset of Endemic Pemphigus Foliaceus. Fogo selvagem (FS), the endemic form of pemphigus foliaceus (PF), is characterized by pathogenic anti-desmoglein 1 (DSG1) autoantibodies. To study the etiology of FS, hybridomas that secrete either IgM or IgG (predominantly IgG1 subclass) autoantibodies were generated from the B cells of eight FS patients and one individual 4 years before FS onset, and the H and L chain V genes of anti-DSG1

2009 Journal of Investigative Dermatology PubMed

185. Tunisian endemic pemphigus foliaceus is associated with the HLA-DR3 gene: anti-desmoglein 1 antibody-positive healthy subjects bear protective alleles. (PubMed)

Tunisian endemic pemphigus foliaceus is associated with the HLA-DR3 gene: anti-desmoglein 1 antibody-positive healthy subjects bear protective alleles. Pemphigus foliaceus is an autoimmune blistering skin disease that partly results from genetic factors, especially human leucocyte antigen (HLA) class II genes.The aim of the study was to determine the HLA DR/DQ markers of susceptibility and protection in the Tunisian endemic form.Genomic DNA from 90 patients with pemphigus foliaceus recruited (...) -positive patients. Secondly, when the HLA DR/DQ allele distribution was studied after dividing patients according to their geographical origin, the southern group, which consisted exclusively of patients with the endemic form of the disease, showed the same associations as the whole pemphigus foliaceus population, particularly with DRB1*03. In the northern group, only the DRB1*04 and DQB1*0301 alleles were found to be associated. Interestingly, anti-desmoglein 1 antibody-positive healthy controls did

2009 British Journal of Dermatology

186. Pemphigus foliaceus with prominent neutrophilic pustules initially presenting as erythroderma. (PubMed)

Pemphigus foliaceus with prominent neutrophilic pustules initially presenting as erythroderma. We report a 64-year-old man who presented with generalized erythroderma and erosions. The erythroderma improved generally as a result of systemic prednisolone treatment. After treatment, however, the patient developed annular erythema with tiny pustules. Histopathology, ELISA and immunoblot analysis showed the disease to be pemphigus foliaceus (PF) with prominent neutrophilic pustules. To our

2009 Clinical & Experimental Dermatology

187. Assessment of vesicular-bullous rash

) Incontinentia pigmenti Bullous ichthyosiform erythroderma (epidermolytic hyper-keratosis) Mastocytosis Bullous arthropod bite reaction Erythema multiforme Stevens-Johnson syndrome Toxic epidermal necrolysis Amyloidosis Hand-foot-mouth disease Congenital syphilis Linear IgA disease Chronic bullous disease of childhood Pemphigus vulgaris Pemphigus foliaceus (fogo selvagem, pemphigus erythematosus) Paraneoplastic pemphigus Bullous pemphigoid Pemphigoid gestationis Mucous membrane pemphigoid (cicatricial (...) eruption with resultant shearing off of the epithelium) or the Asboe-Hansen sign (extension of blister into unblistered skin when pressure is applied to the top of the blister). Berger TG, James WD, Elston DM. Andrew's diseases of the skin, clinical dermatology, 10th edition. WB Saunders; 2008. These signs serve to demonstrate that in some diseases (e.g., pemphigus vulgaris and severe bullous drug eruptions) the extent of blistering is greater than observed by simple inspection. Bullae located

2018 BMJ Best Practice

188. Influence of cigarette smoking on pemphigus:a systematic review and pooled analysis of the literature. (PubMed)

predetermined inclusion and exclusion criteria. Most were case-control studies partly reporting that pemphigus vulgaris and foliaceus occurred less frequently in current and former smokers. Studies also indicated that duration of smoking and number of cigarettes smoked were lower in patients with pemphigus than controls and that remission may be achieved sooner in those who smoke. However, although a generally low prevalence of smoking was demonstrated in patients with pemphigus, which was lower than (...) Influence of cigarette smoking on pemphigus:a systematic review and pooled analysis of the literature. Epidemiological evidence suggests that smoking cigarettes may be beneficial in pemphigus, but no systematic evaluation exists to corroborate this assumption. Therefore, a systematic literature review with pooled data analysis of the smoking status in patients with pemphigus was conducted. Electronic searches using PubMed from inception to November 2017 identified 13 reports meeting

2018 Journal of the European Academy of Dermatology and Venereology

189. Humoral Epitope Spreading in Autoimmune Bullous Diseases (Full text)

and underlined their functional role in disease pathogenesis. A functional ES has been proposed: (1) in bullous pemphigoid patients and correlates with the initial phase of the disease, (2) in pemphigus vulgaris patients with mucosal involvement during the clinical transition to a mucocutaneous form, (3) in endemic pemphigus foliaceus, underlining its role in disease pathogenesis, and (4) in numerous cases of disease transition associated with an intermolecular diversification of immune response. All

2018 Frontiers in immunology PubMed

190. Trichoscopy in Diagnosis of Immunobollous Diseases

Party): tasbeeh salah, Assiut University Study Details Study Description Go to Brief Summary: Autoimmune bullous diseases are a variety of skin diseases that are characterized by the presence of bullae or blisters. Most of these diseases are associated with substantial morbidity and mortality. They are classified according to the site of blister formation into intraepidermal as pemphigus valgaris and foliaceus, and subepidermal as bullous pemphigoid and dermatitis herpetiformis. These lesions (...) of these diseases are associated with substantial morbidity and mortality. They are classified according to the site of blister formation into intraepidermal as pemphigus valgaris and foliaceus, and subepidermal as bullous pemphigoid and dermatitis herpetiformis. These lesions commonly affect the scalp and manifest as blisters, erosions and crustations. Pemphigus is a group of potentially life-threatening intraepidermal vesiculobullous autoimmune diseases that affect the skin and mucous membranes

2018 Clinical Trials

191. Autoimmune skin disease among dermatology outpatients in Botswana: a retrospective review. (PubMed)

erythematosus (SLE) with cutaneous involvement, dermatomyositis, systemic sclerosis, lichen sclerosus, bullous pemphigoid, pemphigus foliaceus, pemphigus vulgaris, and pemphigus vegetans were identified. Demographic data were recorded, and incidence was determined.A total of 262 patients were diagnosed with autoimmune skin disease (4% of all new patients) with an incidence rate of 28.8 (per 1,000,000). Cutaneous lupus was the most common diagnosis with discoid lupus occurring most frequently (12.6 (...) ). The incidence of systemic sclerosis (2.2), morphea (1.6), lichen sclerosus (1.5), SLE with cutaneous involvement (1.3) and dermatomyositis (1.2) was relatively lower. Bullous pemphigoid was the most common bullous disease (3.8). Pemphigus foliaceus (0.9), pemphigus vulgaris (0.6), and pemphigus vegetans (0.2) were observed at a lower rate. At least 19.8% of these patients were also HIV positive.The incidence of autoimmune skin diseases in Botswana is lower compared to other published studies in other

2018 International Journal of Dermatology

192. Update on the cloning of monoclonal anti-desmoglein antibodies from human pemphigus patients: implications for targeted therapy (Full text)

Update on the cloning of monoclonal anti-desmoglein antibodies from human pemphigus patients: implications for targeted therapy Autoantibodies in pemphigus foliaceus (PF) and vulgaris (PV) bind to desmoglein (Dsg) 1 and 3, respectively, and cause loss of keratinocyte adhesion. To characterize the pathogenicity and genetics of such antibodies we have used phage display to isolate monoclonal antibodies (mAbs) from patients. PCR is used to clone the heavy and light chain variable region (...) . Pathogenic antibodies cause typical pemphigus blisters. In both PV and PF patients the heavy chain (VH) genes used for Dsg-binding antibodies are severely restricted. PV and PF patients have both pathogenic and non-pathogenic mAbs. The immunochemical characteristics of the antibodies (including pathogenicity) sort with the VH, not the VL, gene. These monoclonal pathogenic antibodies can be used to screen peptide libraries to find short peptides that block antibody binding. In summary, the antibody

2009 Veterinary dermatology PubMed

193. Biphasic Activation of p38MAPK Suggests That Apoptosis Is a Downstream Event in Pemphigus Acantholysis (Full text)

Biphasic Activation of p38MAPK Suggests That Apoptosis Is a Downstream Event in Pemphigus Acantholysis In pemphigus vulgaris and pemphigus foliaceus (PF), autoantibodies against desmoglein-3 and desmoglein-1 induce epidermal cell detachment (acantholysis) and blistering. Activation of keratinocyte intracellular signaling pathways is emerging as an important component of pemphigus IgG-mediated acantholysis. We previously reported activation of p38 mitogen-activated protein kinase (MAPK (...) ) in response to pathogenic pemphigus vulgaris and PF IgG. Inhibition of p38MAPK blocked pemphigus IgG-induced cytoskeletal reorganization in tissue culture and blistering in pemphigus mouse models. We now extend these observations by demonstrating two peaks of p38MAPK activation in pemphigus tissue culture and mouse models. Administration of the p38MAPK inhibitor SB202190 before PF IgG injection blocked both peaks of p38MAPK phosphorylation and blister formation, consistent with our previous findings

2009 The Journal of biological chemistry PubMed

194. A randomized double-blind trial of intravenous immunoglobulin for pemphigus. (PubMed)

effect of a single cycle of high-dose intravenous immunoglobulin (400, 200, or 0 mg/kg/d) administered over 5 consecutive days in patients relatively resistant to systemic steroids.We evaluated efficacy with time to escape from the protocol as a novel primary end point, and pemphigus activity score, antidesmoglein enzyme-linked immunosorbent assay scores, and safety as secondary end points.We enrolled 61 patients with pemphigus vulgaris or pemphigus foliaceus who did not respond to prednisolone (...) A randomized double-blind trial of intravenous immunoglobulin for pemphigus. Pemphigus is a rare life-threatening intractable autoimmune blistering disease caused by IgG autoantibodies to desmogleins. It has been difficult to conduct a double-blind clinical study for pemphigus partly because, in a placebo group, appropriate treatment often must be provided when the disease flares.A multicenter, randomized, placebo-controlled, double-blind trial was conducted to investigate the therapeutic

2009 Journal of the American Academy of Dermatology

195. Incidence of bullous pemphigoid and pemphigus in Switzerland: a 2-year prospective study. (PubMed)

Incidence of bullous pemphigoid and pemphigus in Switzerland: a 2-year prospective study. Bullous pemphigoid (BP), pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune bullous diseases characterized by the presence of tissue-bound and circulating autoantibodies directed against disease-specific target antigens of the skin. Although rare, these diseases run a chronic course and are associated with significant morbidity and mortality. There are few prospective data on gender (...) - and age-specific incidence of these disorders.Our aims were: (i) to evaluate the incidence of BP and PV/PF in Swiss patients, as the primary endpoint; and (ii) to assess the profile of the patients, particularly for comorbidities and medications, as the secondary endpoint.The protocol of the study was distributed to all dermatology clinics, immunopathology laboratories and practising dermatologists in Switzerland. All newly diagnosed cases of BP and pemphigus occurring between 1 January 2001 and 31

2009 British Journal of Dermatology

196. Pathogenic Epitopes of Autoantibodies in Pemphigus Reside in the Amino-Terminal Adhesive Region of Desmogleins Which Are Unmasked by Proteolytic Processing of Prosequence. (Full text)

with adhesion, only bind the mature form. A pathogenic and three non-pathogenic anti-Dsg1 monoclonal Abs (mAbs) isolated from a pemphigus foliaceus (PF) patient, were used for immunoprecipitation and ELISA of recombinant precursor and mature Dsg1. The pathogenic Ab binds mature Dsg1, whereas non-pathogenic Abs bind either only the precursor or both the precursor and mature Dsg1. Competition ELISA showed that the majority of PF sera target the same or nearby epitopes defined by the pathogenic anti-Dsg1 mAb (...) Pathogenic Epitopes of Autoantibodies in Pemphigus Reside in the Amino-Terminal Adhesive Region of Desmogleins Which Are Unmasked by Proteolytic Processing of Prosequence. Pemphigus targets desmogleins (Dsgs), which are thought to be synthesized as inactive precursor proteins with prosequences that are cleaved by substilisin-like proprotein convertases, such as furin, to yield mature adhesive molecules. We hypothesized that some pemphigus pathogenic antibodies (Abs), which presumably interfere

2009 Journal of Investigative Dermatology PubMed

197. Trichoscopy - a useful tool in the preliminary differential diagnosis of autoimmune bullous diseases. (PubMed)

was to determine the value of trichoscopy in the differential diagnosis of pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, and dermatitis herpetiformis.Trichoscopy was used to evaluate scalp lesions in 68 patients (26 with pemphigus vulgaris, 17 with pemphigus foliaceus, 17 with bullous pemphigoid, and 8 with dermatitis herpetiformis). The working magnification was 20-fold and 70-fold.The most frequent trichoscopic feature of autoimmune bullous diseases was extravasations. They occurred in 76.9 (...) % of patients with pemphigus vulgaris, 70.6% of patients with pemphigus foliaceus, 76.5% of patients with bullous pemphigoid, and 100% of patients with dermatitis herpetiformis. Yellow hemorrhagic crusts occurred in, respectively, 73.1%, 70.6%, 64.7%, and 35.5% of the cases. Yellow diffuse scaling and tubular scaling occurred more frequently in pemphigus foliaceus (52.9% and 41.2%, respectively). Clustered dotted vessels were characteristic for dermatitis herpetiformis (5/8, 62.5%). Dotted vessels

2017 International Journal of Dermatology

198. Determining the Incidence of Pneumocystis Pneumonia in Patients With Autoimmune Blistering Diseases Not Receiving Routine Prophylaxis. (Full text)

for patients with autoimmune blistering disease in Germany, Italy, Singapore, Israel, and the Netherlands. Patients had a confirmed diagnosis of pemphigus vulgaris/foliaceus, bullous pemphigoid, epidermolysis bullosa acquisita, mucous membrane pemphigoid/cicatricial pemphigoid, or anti-p200 pemphigoid.To determine the incidence of PCP defined as patients with the International Classification of Diseases, Ninth Revision (ICD-9), code 136.3, for PCP, or free text documentation of PCP occurring based

2017 JAMA dermatology (Chicago, Ill.) PubMed

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