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Pemphigus Foliaceus

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161. Pemphigus, Paraneoplastic (Diagnosis)

(desmoglein 3, 130-kd) and pemphigus foliaceus (desmoglein 1, 160-kd), as well as several others, including epiplakin. [ ] Antibodies are typically IgG, although IgA has been reported. [ ] In a 2011 review, Czernik et al . summarize the increasing role of cellular immunity in paraneoplastic pemphigus, evidenced by lesional mononuclear cells and elevated IL-6 levels in sera from paraneoplastic pemphigus patients. [ ] Patients with paraneoplastic pemphigus without identifiable autoantibodies have been (...) Pemphigus, Paraneoplastic (Diagnosis) Paraneoplastic Pemphigus: Background, Pathophysiology, Etiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTA2NDQ1Mi1vdmVydmlldw== processing > Paraneoplastic Pemphigus

2014 eMedicine.com

162. Pemphigus, IgA (Diagnosis)

with having IgA-pemphigus foliaceus (IgA-PF) or IgA-pemphigus vulgaris (IgA-PV), respectively. All other cases not meeting these criteria are diagnosed as unclassified IAD. IgA pemphigus has not been described in animals. Although the term IgA pemphigus has not been established in the veterinary literature, IgA deposition around epidermal cells has been detected by direct immunofluorescence in animals affected with pemphigus foliaceus. Medscape articles on other variants of pemphigus include (...) , Hashimoto T, et al. Autoantibodies against desmocollins in European patients with pemphigus. Clin Exp Dermatol . 2009 Dec. 34(8):898-903. . Chan LS, Vanderlugt CJ, Hashimoto T, et al. Epitope spreading: lessons from autoimmune skin diseases. J Invest Dermatol . 1998 Feb. 110(2):103-9. . Chorzelski TP, Beutner EH, Kowalewski C, et al. IgA pemphigus foliaceus with a clinical presentation of pemphigus herpetiformis. J Am Acad Dermatol . 1991 May. 24(5 Pt 2):839-44. . Erdag G, Qureshi HS, Greer KE

2014 eMedicine.com

163. Pemphigus Vulgaris (Diagnosis)

of other diseases. The outlook is worse in older patients and in patients with extensive disease. Prognosis is usually better in childhood than in adulthood. A few rare cases of pemphigus vulgaris transitioning to pemphigus foliaceus have been reported. Previous Next: Patient Education Minimize trauma to the skin because the patient's skin is fragile both from the disease and from the use of topical and systemic steroids. The patient's understanding of the disease and education about pemphigus vulgaris (...) of the Pathogenesis of Pemphigus and Pemphigoid Meeting in Munich, September 2016. J Invest Dermatol . 2017 Jun. 137 (6):1199-1203. . Mentink LF, de Jong MC, Kloosterhuis GJ, Zuiderveen J, Jonkman MF, Pas HH. Coexistence of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. Br J Dermatol . 2007 Apr. 156(4):635-41. . Bhol K, Mohimen A, Ahmed AR. Correlation of subclasses of IgG with disease activity in pemphigus vulgaris. Dermatology . 1994. 189 Suppl 1

2014 eMedicine.com

164. Pemphigus Erythematosus (Diagnosis)

Updated: Mar 02, 2018 Author: Rakesh Bharti, MD, MBBS; Chief Editor: William D James, MD Share Email Print Feedback Close Sections Sections Pemphigus Erythematosus Overview Background The various types of pemphigus include pemphigus erythematosus, pemphigoid, pemphigus vegetans, pemphigus vulgaris, and . Pemphigus erythematosus, also known as Senear-Usher syndrome, is an overlap syndrome with features of lupus erythematosus (LE) and pemphigus foliaceus. Pemphigus is demonstrated by acantholysis (...) and immunoglobulin deposits in the interkeratinocyte substance (see the image below). Direct immunofluorescence microscopy performed on epithelial biopsy specimen obtained from a patient with pemphigus vulgaris detects immunoglobulin G deposits at the epithelial cell surfaces. Pérez-Pérez et al recently hypothesized that pemphigus erythematosus is a multiple autoimmune disease. [ ] High doses of UV light are suggested to be the cause of cleavage of the desmoglein-1 ectodomain. As in cases of pemphigus foliaceus

2014 eMedicine.com

165. Pemphigus Herpetiformis (Diagnosis)

affected with pemphigus herpetiformis. At least 2 cases of pemphigus herpetiformis have been reported to occur in association with lung cancer. Whether this association was coincidental is not clear. [ , ] In addition, pemphigus herpetiformis has been associated with prostate cancer development in one case. [ ] Previous References Joly P, Litrowski N. Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clin Dermatol . 2011 Jul-Aug. 29(4):432-6. . Laws PM, Heelan K, Al (...) reports. Dermatologica Sinica . 2010 Mar. 28 (1):36-39. Robinson ND, Hashimoto T, Amagai M, Chan LS. The new pemphigus variants. J Am Acad Dermatol . 1999 May. 40 (5 Pt 1):649-71;672-3. . Jablonska S, Chorzelski TP, Beutner EH, Chorzelska J. Herpetiform pemphigus, a variable pattern of pemphigus. Int J Dermatol . 1975 Jun. 14 (5):353-9. . Cunha PR, Jiao D, Bystryn JC. Simultaneous occurrence of herpetiform pemphigus and endemic pemphigus foliaceus (fogo selvagem). Int J Dermatol . 1997 Nov. 36 (11

2014 eMedicine.com

166. Clinical and histological characterization of oral pemphigus lesions in patients with skin diseases: a cross sectional study from Sudan. (PubMed)

of diagnosis. IHC was used to confirm the diagnosis. Location, size, and pain of oral lesions were used to measure the oral disease activity.Twenty-one patients were diagnosed with pemphigus vulgaris (PV), 19 of them (mean age: 43.0; range: 20-72 yrs) presented with oral manifestations. Pemphigus foliaceus was diagnosed in one patient. In PV, female: male ratio was 1.1:1.0. Buccal mucosa was the most commonly affected site. Exclusive oral lesions were detected in 14.2% (3/21). In patients who experienced (...) Clinical and histological characterization of oral pemphigus lesions in patients with skin diseases: a cross sectional study from Sudan. Pemphigus is a rare group of life-threatening mucocutaneous autoimmune blistering diseases. Frequently, oral lesions precede the cutaneous ones. This study aimed to describe clinical and histological features of oral pemphigus lesions in patients aged 18 years and above, attending outpatient's facility of Khartoum Teaching Hospital - Dermatology Clinic, Sudan

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2013 BMC Oral Health

167. Afro-Caribbean pemphigus: epidemiological data from a 5-year prospective study on the island of Guadeloupe (French West Indies). (PubMed)

to the Dermatology Department or secondarily referred by their private practice dermatologist once identified by the computerized databases of the Guadeloupian pathology laboratories.World-population-standardized incidence was 6.96 (95% CI: 3.41-10.52) for pemphigus vulgaris and 3.75 (95% CI: 1.12-6.39) for pemphigus foliaceus. Patients usually live in the rural countryside, whereas 75% of the population of Guadeloupe Island live in an urban environment.We report a high incidence of autoimmune pemphigus (...) Afro-Caribbean pemphigus: epidemiological data from a 5-year prospective study on the island of Guadeloupe (French West Indies). There are no reported epidemiological data regarding autoimmune pemphigus in the Afro-Caribbean population.To present the epidemiology of autoimmune pemphigus on the island of Guadeloupe (French West Indies, 400,736 inhabitants, mostly black Caribbean of African European descent).Five-year prospective study. Inclusion of the incident cases when directly referred

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2013 International Journal of Dermatology

168. Efficacy and Safety of Ofatumumab in Treatment of Pemphigus Vulgaris

methods are not applicable in Japan: oral contraceptives with progestogen alone, injectable progesterone, levonorgestrel implants, estrogenic vaginal ring, percutaneous contraceptive patches, vaginal spermicidal foam, gel, film, and cream French subjects: In France, a subject will be eligible for inclusion in this study only if either affiliated to or a beneficiary of a social security category. Exclusion Criteria: Diagnosis of pemphigus foliaceus, paraneoplastic pemphigus, or other autoimmune (...) Efficacy and Safety of Ofatumumab in Treatment of Pemphigus Vulgaris Efficacy and Safety of Ofatumumab in Treatment of Pemphigus Vulgaris - 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. Efficacy and Safety

2013 Clinical Trials

169. CTLA4CT60 gene polymorphism is not associated with differential susceptibility to pemphigus foliaceus (PubMed)

CTLA4CT60 gene polymorphism is not associated with differential susceptibility to pemphigus foliaceus Pemphigus foliaceus is an organ-specific autoimmune disease characterized by autoantibodies against the extracellular region of desmoglein 1, a protein that mediates intercellular adhesion in desmosomes. Cytotoxic T-lymphocyte-associated protein 4 (CTLA-4) is a key negative regulator of the T cell immune response, playing an important role in T cell homeostasis and maintenance of peripheral (...) tolerance. Polymorphisms in the CTLA4 gene have been associated with autoimmune diseases and the functional CT60 single nucleotide polymorphism (rs3087243, also named 6230G > A) has been proposed to be a casual variant in several of these diseases. The aim of this study was to ascertain whether this polymorphism is associated with inter-individual variation in susceptibility to pemphigus foliaceus. The population sample in this case-control association study comprised 248 patient and 367 controls. We

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2010 Genetics and molecular biology

170. IgE, IgM and IgG4 anti-desmoglein 1 autoantibody profile in endemic pemphigus foliaceus (Fogo Selvagem) (PubMed)

IgE, IgM and IgG4 anti-desmoglein 1 autoantibody profile in endemic pemphigus foliaceus (Fogo Selvagem) 21191415 2011 05 31 2018 11 13 1523-1747 131 4 2011 Apr The Journal of investigative dermatology J. Invest. Dermatol. IgE, IgM, and IgG4 anti-desmoglein 1 autoantibody profile in endemic pemphigus foliaceus (fogo selvagem). 985-7 10.1038/jid.2010.403 Qian Ye Y Prisayanh Phillip P Andraca Eugenio E Qaqish Bahjat F BF Aoki Valeria V Hans-Filhio Gunter G Rivitti Evandro A EA Diaz Luis A LA (...) AR NIAMS NIH HHS United States K01 AR056378 AR NIAMS NIH HHS United States Letter Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't 2010 12 30 United States J Invest Dermatol 0426720 0022-202X 0 Autoantibodies 0 DSG1 protein, human 0 Desmoglein 1 0 Immunoglobulin G 0 Immunoglobulin M 37341-29-0 Immunoglobulin E IM Autoantibodies blood Desmoglein 1 immunology Environment Humans Immunoglobulin E blood Immunoglobulin G blood Immunoglobulin M blood Pemphigus epidemiology

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2010 The Journal of investigative dermatology

171. Human eyelid meibomian glands and tarsal muscle are recognized by autoantibodies from patients affected by a new variant of endemic pemphigus foliaceus in El-Bagre, Colombia, South America. (PubMed)

Human eyelid meibomian glands and tarsal muscle are recognized by autoantibodies from patients affected by a new variant of endemic pemphigus foliaceus in El-Bagre, Colombia, South America. Previously, we described a new variant of endemic pemphigus foliaceus (EPF) in Colombia, South America (El Bagre-EPF).Continuing our characterization of this variant of EPF, we now focus on one of our previously reported clinical findings: the presence of ocular lesions. These ocular lesions are seen (...) and ocular lesions, 5 patients with sporadic (nonendemic) pemphigus foliaceus, and 20 healthy control subjects (10 from the El Bagre-EPF endemic area and 10 from nonendemic areas). We used fluorescein isothiocyanate conjugated goat antiserum to human total IgG/IgA/IgM as a secondary antibody. In addition, we used fluorescein isothiocyanate conjugated antibodies to human fibrinogen, albumin, IgG, IgE, C1q, and C3, Texas Red (Rockland Immunochemicals, Inc, Gilbertsville, PA), Alexa Fluor 555, or Alexa

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2010 Journal of American Academy of Dermatology

172. Immune response towards amino-terminus of desmoglein 1 prevails across different activity stages in nonendemic pemphigus foliaceus. (PubMed)

Immune response towards amino-terminus of desmoglein 1 prevails across different activity stages in nonendemic pemphigus foliaceus. Pemphigus foliaceus (PF) is a blistering skin disease mediated by antibodies to desmoglein (Dsg) 1. The two major subtypes are nonendemic and endemic PF. A previous study in endemic PF demonstrated that changes in antibody epitope could modulate disease relapse and remission.To characterize the frequency of immunoreactivity to various Dsg1 extracellular (EC

2010 British Journal of Dermatology

173. Five Japanese cases of anti-Dsg1 antibody positive and anti-Dsg3 antibody negative pemphigus with oral lesions. (PubMed)

Five Japanese cases of anti-Dsg1 antibody positive and anti-Dsg3 antibody negative pemphigus with oral lesions. Oral mucosal lesions develop in pemphigus vulgaris, but not in pemphigus foliaceus. This clinical phenomenon is explained by the 'desmoglein (Dsg) compensation theory'. Dsg3 and Dsg1 are major autoantigens for pemphigus vulgaris and pemphigus foliaceus, respectively. Dsg3 is overexpressed and Dsg1 is weakly expressed on the oral mucosa. Thus, on the oral mucosa, suppression of Dsg3 (...) clinical phenotypes in our patients were due to a different immunological profile from that in classical pemphigus, we examined the reactivity of the patient sera by immunoprecipitation-immunoblotting analysis using five Dsg1/Dsg2 domain-swapped molecules.The sera of two patients who had only oral lesions tended to react with the extracellular (EC) 5 domain of Dsg1, the domain that is considered nonpathogenic in classical pemphigus foliaceus. Sera of three patients with mucocutaneous lesions reacted

2012 British Journal of Dermatology

174. Enrichment of total serum IgG4 in pemphigus patients. (PubMed)

Enrichment of total serum IgG4 in pemphigus patients. Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are potentially fatal blistering diseases caused by autoantibodies targeting desmoglein (Dsg) adhesion proteins. Previous studies have shown an IgG4 > IgG1 predominance of anti-Dsg antibodies in pemphigus; however, no studies have examined total serum IgG4 levels in pemphigus. IgG4 is induced by chronic antigen stimulation, which could occur with persistent skin blistering and potentially (...) fractions.Dsg-specific autoantibodies are significantly enriched in IgG4, which may explain the enrichment of total serum IgG4 in some patients with pemphigus. By preferentially targeting autoimmune rather than beneficial immune antibodies, IgG4-targeted therapies may offer safer treatment options for pemphigus.© 2012 The Authors. BJD © 2012 British Association of Dermatologists 2012.

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2012 British Journal of Dermatology

175. The IgG "Lupus-Band" Deposition Pattern of Pemphigus Erythematosus: Association With the Desmoglein 1 Ectodomain as Revealed by 3 Cases. (PubMed)

The IgG "Lupus-Band" Deposition Pattern of Pemphigus Erythematosus: Association With the Desmoglein 1 Ectodomain as Revealed by 3 Cases. Pemphigus foliaceus is an autoimmune skin disease characterized by subcorneal blistering and IgG antibodies directed against desmoglein 1. In the skin, these antibodies deposit intraepidermally. On rare occasions,an additional “lupus band” of granular depositions of IgG and complement is seen along the epidermal basement membrane zone. This combined pattern (...) has been connected with a variant of pemphigus foliaceus named pemphigus erythematosus.We describe 3 pemphigus foliaceus cases in which phototherapy was administered after a misdiagnosis of psoriasis. This treatment resulted in a flare of skin lesions. Direct immunofluorescence of skin biopsy specimens that were obtained several weeks later demonstrated intraepidermal and granular basement membrane zone depositions. The basement membrane zone depositions consisted of IgG, complement

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2012 Archives of Dermatology

176. IgG induced clustering of desmogleins 1 and 3 in pemphigus patient skin fits with the desmoglein non-assembly depletion hypothesis. (PubMed)

and desmosomal adhesion molecules in skin biopsies of 18 patients with pemphigus vulgaris (PV) and 10 with pemphigus foliaceus (PF) by double staining immunofluorescence. The effect of IgG on desmosomal proteins was studied in an in vitro skin model.In PF skin Dsg1, but not Dsg3, was aberrantly distributed in the same partly granular pattern as the IgG. Vice versa, in skin of PV patients with anti-Dsg3 antibodies, Dsg3, but not Dsg1, colocalized with the granular IgG. Plakoglobin also coclustered with IgG (...) IgG induced clustering of desmogleins 1 and 3 in pemphigus patient skin fits with the desmoglein non-assembly depletion hypothesis. In pemphigus circulating IgG is present with the desmosomal cadherins desmoglein (Dsg) 1 and 3. In the epidermis of patients, this IgG deposits in a pattern that is often partly granular and does not reflect the normal Dsg distribution.To understand why the IgG deposits in a granular pattern in the skin of patients with pemphigus.We analysed the distribution of IgG

2011 British Journal of Dermatology

177. Spectrum of autoantibodies other than anti-desmoglein in pemphigus patients. (PubMed)

105 pemphigus foliaceus (PF) patients, 51 pemphigus vulgaris (PV) patients and 50 controls. Both indirect immunofluorescence assay and ELISA were used to assess the presence of autoantibodies related to connective tissue diseases, autoimmune hepatitis, vasculitis, rheumatoid arthritis, coeliac disease, diabetes and thyroiditis.Significant difference was observed between the three groups for anti-thyroglobulin antibodies in the pemphigus foliaceus group (18% vs. 4%, P=0.03). A significantly higher (...) Spectrum of autoantibodies other than anti-desmoglein in pemphigus patients. Pemphigus is a life-threatening autoimmune blistering disease mediated by autoantibodies against adhesion molecule of the skin. Its concurrence with systemic and organ-specific autoimmune disease was described in case reports.To evaluate the presence of a broad spectrum of organ-specific and non-organ-specific autoantibodies other than anti-desmoglein antibodies in pemphigus patients.Serum samples were obtained from

2011 Journal of the European Academy of Dermatology and Venereology

178. Interventions for pemphigus vulgaris and pemphigus foliaceus. (PubMed)

Interventions for pemphigus vulgaris and pemphigus foliaceus. A range of interventions have been described for treatment of pemphigus, however the optimal therapeutic strategy has not been established.To assess the efficacy and safety of all interventions used in the management of pemphigus vulgaris and pemphigus foliaceus.We searched the Cochrane Skin Group Specialised Register (October 2008), The Cochrane Central Register of Controlled Trials (The Cochrane Library Issue 4, 2008), MEDLINE (...) included studies.Eleven studies with a total of 404 participants (337 pemphigus vulgaris, 27 pemphigus foliaceus and 40 not specified ) were identified. The quality of included studies was not high, the majority of studies did not report allocation concealment, and power was limited by very small sample sizes. Interventions assessed included prednisolone dose regimen, pulsed dexamethasone, azathioprine, cyclophosphamide, cyclosporine, dapsone, mycophenolate, plasma exchange, topical epidermal growth

2009 Cochrane database of systematic reviews (Online)

179. Homologous regions of autoantibody heavy chain complementarity-determining region 3 (H-CDR3) in patients with pemphigus cause pathogenicity (PubMed)

these antibodies, we characterized Dsg-specific mAbs cloned by phage display from 3 patients with pemphigus vulgaris and 2 with pemphigus foliaceus. Variable heavy chain gene usage was restricted, but similar genes were used for both pathogenic and nonpathogenic mAbs. However, the heavy chain complementarity-determining region 3 (H-CDR3) of most pathogenic, but not nonpathogenic, mAbs shared an amino acid consensus sequence. Randomization of the H-CDR3 and site-directed mutagenesis indicated that changes (...) Homologous regions of autoantibody heavy chain complementarity-determining region 3 (H-CDR3) in patients with pemphigus cause pathogenicity Pemphigus is a life-threatening autoimmune disease in which antibodies specific for desmogleins (Dsgs) cause loss of keratinocyte cell adhesion and blisters. In order to understand how antibodies cause pathogenicity and whether there are commonalities among antibodies in different patients that could ultimately be used to target specific therapy against

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2010 The Journal of clinical investigation

180. Correlation of immunological profile with phenotype and disease outcome in pemphigus. (PubMed)

and immunopathological data were collected from 40 patients. Patients sera were characterized by indirect immunofluorescence and enzyme-linked immunosorbent assay (ELISA). Epidemiological and clinical findings were comparable to other series. Mortality during follow-up was 0% and 6% in pemphigus foliaceus and vulgaris, respectively. Importantly, higher indirect immunofluorescence titres and anti-desmoglein 3 ELISA values of samples from untreated patients correlated significantly with a potentially worse clinical (...) Correlation of immunological profile with phenotype and disease outcome in pemphigus. There has been no previous clinical-immunological study of pemphigus in Spain. The aim of this study was to perform a retrospective analysis of pemphigus patients who had been followed for a period of 18 years in our centre. We characterized the autoantibody response, compared diagnostic assays and correlated the immunobiological data with phenotype and prognosis. Clinical, epidemiological

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2010 Acta Dermato-Venereologica

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