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Pemphigus Foliaceus

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161. Pemphigus Vulgaris (Follow-up)

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 1:85-9. . Wilson CL, Wojnarowska F, Dean D, Pasricha JS. IgG subclasses in pemphigus in Indian and UK populations. Clin Exp Dermatol . 1993 May. 18(3):226-30. . Fitzpatrick RE, Newcomer VD. The correlation (...) lesion. Oral Surg Oral Med Oral Pathol . 1974 Sep. 38(3):382-7. . Simon DG, Krutchkoff D, Kaslow RA, Zarbo R. Pemphigus in Hartford County, Connecticut, from 1972 to 1977. Arch Dermatol . 1980 Sep. 116(9):1035-7. . Bastuji-Garin S, Souissi R, Blum L, et al. Comparative epidemiology of pemphigus in Tunisia and France: unusual incidence of pemphigus foliaceus in young Tunisian women. J Invest Dermatol . 1995 Feb. 104(2):302-5. . Hietanen J, Salo OP. Pemphigus: an epidemiological study of patients

2014 eMedicine.com

162. 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

163. Afro-Caribbean pemphigus: epidemiological data from a 5-year prospective study on the island of Guadeloupe (French West Indies). Full Text available with Trip Pro

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

2013 International Journal of Dermatology

164. Clinical and histological characterization of oral pemphigus lesions in patients with skin diseases: a cross sectional study from Sudan. Full Text available with Trip Pro

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

2013 BMC Oral Health

165. Enrichment of total serum IgG4 in pemphigus patients. Full Text available with Trip Pro

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.

2012 British Journal of Dermatology

166. The IgG "Lupus-Band" Deposition Pattern of Pemphigus Erythematosus: Association With the Desmoglein 1 Ectodomain as Revealed by 3 Cases. Full Text available with Trip Pro

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

2012 Archives of Dermatology

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

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

168. Spectrum of autoantibodies other than anti-desmoglein in pemphigus patients. (Abstract)

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

169. IgG induced clustering of desmogleins 1 and 3 in pemphigus patient skin fits with the desmoglein non-assembly depletion hypothesis. Full Text available with Trip Pro

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

170. IgE, IgM and IgG4 anti-desmoglein 1 autoantibody profile in endemic pemphigus foliaceus (Fogo Selvagem) Full Text available with Trip Pro

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

2010 The Journal of investigative dermatology

171. CTLA4CT60 gene polymorphism is not associated with differential susceptibility to pemphigus foliaceus Full Text available with Trip Pro

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

2010 Genetics and molecular biology

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

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. 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. Full Text available with Trip Pro

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

2010 Journal of American Academy of Dermatology

174. Polyvalent immunoglobulins – Part 1: A rapid review

. The remaining 63 reviews were on a wide range of other indications, for which an overview is offered in 2.3.4. High quality SRs were present for the most well-known indications whereas there were few high quality studies for more recent indications such as dermatological diseases (Pemphigus of Pemphigus vulgaris, foliculae, urticaria). 20 Immunoglobulines KCE Report 327 Figure 2 – PRISMA flowchart of general literature search for Systematic Reviews (Internal Scoping Phase) KCE Report 327 Immunoglobulines 21

2020 Belgian Health Care Knowledge Centre

175. 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

176. European Guidelines (S1) on the use of high-dose intravenous immunoglobulin in dermatology

foliaceus, mucous membrane pemphigoid [16] and epidermolysis 8 bullosa acquisita [41]. However, the use of IVIg may also be indicated in severe forms of bullous pemphigoid [42], linear IgA disease, IgA pemphigus or paraneoplastic pemphigus. 2. Timing of treatment: On the basis of the scientific evidence available, the use of IVIg cannot be recommended as a first-line treatment. However, contraindications to standard immunosuppressive therapy (e.g., aseptic bone necrosis, poorly controlled diabetes (...) . Long-term treatment advisable? Dermatomyositis: Dermatomyositis is the condition in which the highest level of evidence exists for treatment with IVIg besides pemphigus vulgaris [6]. There are numerous individual case reports and small case series [7] as well as a double-blind, placebo-controlled crossover study, which demonstrate the efficacy of IVIg [8]. The following criteria were drawn up by the European Guidelines working group: 1. Indications: All severe forms of dermatomyositis, inclusion

2019 European Dermatology Forum

177. Homologous regions of autoantibody heavy chain complementarity-determining region 3 (H-CDR3) in patients with pemphigus cause pathogenicity Full Text available with Trip Pro

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

2010 The Journal of clinical investigation

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

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

179. Correlation of immunological profile with phenotype and disease outcome in pemphigus. Full Text available with Trip Pro

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

2010 Acta Dermato-Venereologica

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

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

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