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Pemphigus Foliaceus

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181. Serum plakophilin-3 autoreactivity in paraneoplastic pemphigus. (Abstract)

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

182. Autoimmune skin disease among dermatology outpatients in Botswana: a retrospective review. Full Text available with Trip Pro

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

183. Humoral Epitope Spreading in Autoimmune Bullous Diseases Full Text available with Trip Pro

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

184. 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

185. Pemphigoid

:1681-7. 31 Kirtschig G, Middleton P, Bennett C et al. Interventions for bullous pemphigoid. Cochrane Database Syst Rev 2010; 6:10. 32 Daniel BS, Borradori L, Hall RP 3rd, Murrell DF. Evidence-based management of bullous pemphigoid. Dermatol Clin 2011; 29:613-20. 33 Singh S. Evidence-based treatments for pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid: a systematic review. Indian J Dermatol Venereol Leprol 2011; 77:456-69. 34 Roujeau JC, Guillaume JC, Morel P et al. Plasma exchange (...) activities outside the submitted work 1 Board membership No No No No 2 Consultancy No No No No 3 Employment No No No No 4 Expert testimony No No No No 5 Grants/grants pending Dompé S.p.A. research grant “Possible role of IL-8 in pemphigus pathogenesis” (2011-2013) No No No 6 Payment for lectures including service on speakers bureaus No No No No 7 Payment for manuscript preparation No No No No 8 Patents (planned, pending or issued) No No No No 9 Royalties No No No No 10 Payment for development

2014 European Dermatology Forum

188. Determining the Incidence of Pneumocystis Pneumonia in Patients With Autoimmune Blistering Diseases Not Receiving Routine Prophylaxis. Full Text available with Trip Pro

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.)

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

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

191. Correlation of Serum Levels of IgE Autoantibodies Against BP180 With Bullous Pemphigoid Disease Activity. (Abstract)

with BP underwent clinical evaluation with the Bullous Pemphigoid Disease Activity Index (BPDAI); and 36 patients with BP without anti-BP180 NC16A IgG reactivity underwent evaluation of the diagnostic importance of serum anti-BP180 IgE. In addition, 49 age-matched control individuals with noninflammatory dermatoses, 127 controls undergoing allergy testing for IgE levels, and 30 controls with pemphigus vulgaris or pemphigus foliaceus were included for comparison. Patients were seen at a university

2016 JAMA dermatology (Chicago, Ill.)

192. Clinical and immunological studies of 49 cases of various types of intercellular IgA dermatosis and 13 cases of classical subcorneal pustular dermatosis examined at Kurume University. Full Text available with Trip Pro

, we selected 49 cases of various types of intercellular IgA dermatosis (IAD) and 13 cases of classical subcorneal pustular dermatosis (SPD), for which sera and information were available. We studied these cases clinically and immunologically.There were 17 SPD-type IAD, 12 intraepidermal neutrophilic IgA dermatosis (IEN)-type IAD, two IgA-pemphigus vegetans, four IgA-pemphigus foliaceus, six IgA-pemphigus vulgaris and eight unclassified IAD cases. There was no sex predominance, and the average age

2016 British Journal of Dermatology

193. Non-infectious environmental antigens as a trigger for the initiation of an autoimmune skin disease. Full Text available with Trip Pro

Non-infectious environmental antigens as a trigger for the initiation of an autoimmune skin disease. Pemphigus represents a group of organ specific autoimmune blistering disorders of the skin mediated by pathogenic autoantibodies with well-defined antigenic targets. While most of these diseases are sporadic, endemic forms of disease do exist. The endemic form of pemphigus foliaceus (also known as fogo selvagem, FS) exhibits epidemiological features that suggest exposure to hematophagous insect

2016 Autoimmunity reviews

194. Register of Autoimmune Bullous Dermatoses

collection Outcome Measures Go to Primary Outcome Measures : autoimmune bullous dermatosis according to physical examination, skin biopsy and autoantibodies detection in blood sample [ Time Frame: Day 0 ] Each autoimmune bullous dermatosis is classified according one of the following diagnoses : 1/ bullous pemphigoid, 2/ Mucous membrane pemphigoid, 3/ Pemphigoid gestationis, 4/ Epidermolysis bullosa acquisita, 5/ Linear IgA bullous dermatosis, 6/ pemphigus (vulgaris or foliaceus) This classification (...) both for bullous pemphigoid and pemphigus which are the most frequent diseases. Condition or disease Intervention/treatment Autoimmune Bullous Dermatoses Other: Data collection Detailed Description: The aim of the study is the creation of a register of patients suffering from autoimmune bullous dermatoses to quickly identify patients who can be included in clinical trials or retrospective epidemiological studies Study Design Go to Layout table for study information Study Type : Observational Actual

2016 Clinical Trials

195. Autoimmune Blistering Diseases Study

, University of Pennsylvania Study Details Study Description Go to Brief Summary: Pemphigus and bullous pemphigoid (BP) are severe autoimmune blistering diseases (AIBD) that pose a critical need for new therapeutic approaches. Clinical trials in pemphigus and BP will require the availability of validated disease severity measures that can be used to define primary outcomes. Condition or disease Intervention/treatment Pemphigus Vulgaris Pemphigus Foliaceus Bullous Pemphigoid Other: Questionnaires Detailed (...) Adult) Sexes Eligible for Study: All Accepts Healthy Volunteers: No Sampling Method: Non-Probability Sample Study Population Adult patients over the age of 18 who have been diagnosed with pemphigus vulgaris, pemphigus foliaceus or bullous pemphigoid. Criteria Inclusion Criteria: Gender/Age: Males or females above 18 years old Diagnosis: pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid Subjects able to give informed consent Exclusion Criteria: Patients who are under age 18 years. Patients

2016 Clinical Trials

196. A review of case-control studies on the risk factors for the development of autoimmune blistering diseases. (Abstract)

A review of case-control studies on the risk factors for the development of autoimmune blistering diseases. Autoimmune blistering diseases (AIBD) are a group of rare but potentially fatal diseases characterized by the production of autoantibodies directed against the structural proteins of the skin. Much has been published on the clinical manifestation and interventional options for AIBD, especially on the more common subtypes such as bullous pemphigoid, pemphigus vulgaris (PV) and pemphigus (...) foliaceus (PF). However, the aetiology of AIBD remains unknown. We aim to provide an overview of published case-control studies focussing on the non-genetic aetiological factors of bullous pemphigoid, PV and/or PF. The relevant studies were appraised for their validity and results. Our results showed that a large proportion of the studies had inconclusive results due to compromised study methodologies. Moreover, there were no identified case-control studies that investigated the possible associations

2015 Journal of the European Academy of Dermatology and Venereology

197. Large-Scale Electron Microscopy Maps of Patient Skin and Mucosa Provide Insight into Pathogenesis of Blistering Diseases. Full Text available with Trip Pro

resolution in a 'Google Earth' approach. We studied the life-threatening human autoimmune blistering disease pemphigus, using nanotomy. The pathomechanism of cell-cell separation (acantholysis) that underlies the blistering is poorly understood. Ultrastructural examination of pemphigus tissue revealed previously unreported findings: (i) the presence of double-membrane structures between cells in all pemphigus types; (ii) the absence of desmosomes around spontaneous blisters in pemphigus foliaceus (PF

2015 Journal of Investigative Dermatology

198. Allelic and copy-number variations of FcγRs affect granulocyte function and susceptibility for autoimmune blistering diseases. (Abstract)

expression, and consequently, bullous pemphigoid (BP) disease risk. We compared this to findings in pemphigus vulgaris/foliaceus (PV/PF), two Fc-independent AIBDs. Our results indicate that both allele and copy number variation of FcγR genes affect FcγR mRNA expression and reactive oxygen species (ROS) release by granulocytes. Susceptibility of BP was associated with FcγR genotypes that led to a decreased ROS release by neutrophils, indicating an unexpected protective role for these cells. BP and PV/PF

2015 Journal of Autoimmunity

199. B-cell activating factor detected on both naïve and memory B-cells in bullous pemphigoid. (Abstract)

, pemphigus foliaceus and bullous pemphigoid (BP), were analysed by flow cytometry. Compared with healthy controls (HC), BAFF expression on naïve and memory B cells increased significantly in BP. No difference in BAFF receptor expression in naïve and memory B cells was shown among all study groups. Furthermore, BAFF expression in both naïve and memory B cells of BP, but not HC, was detected by confocal microscopic analysis. These results implied that BAFF expressed by B cells may play a pathogenic role (...) B-cell activating factor detected on both naïve and memory B-cells in bullous pemphigoid. B-cell activating factor (BAFF), an important immune regulatory cytokine, is involved in development of autoimmune diseases. Although BAFF is expressed in various cells, including dendritic cells (DCs) and monocytes, BAFF expression on B cells has not been well documented. In the present study, BAFF molecules on DCs and naïve and memory B cells in autoimmune bullous diseases, including pemphigus vulgaris

2014 Experimental Dermatology

200. Treatment of severe autoimmune blistering skin diseases with combination of protein A immunoadsorption and rituximab: a protocol without initial high dose or pulse steroid medication. (Abstract)

.) with such diseases: 6 pts. with pemphigus vulgaris, 3 pts. with bullous pemphigoid, 3 pts. with mucous membrane pemphigoid (MMP), one being anti-laminin-332-MMP (AL332-MMP), 2 pts. with pemphigus foliaceus and 1 pt. with epidermolysis bullosa acquisita (EBA). Patients were treated with a combination of protein A immunoadsorption (PAIA, 3-21 treatments) and rituximab (3-6 treatments) in addition to low dose conventional immunosuppression.All patients showed rapid clinical improvement starting within the first 4 (...) weeks and decline of circulating autoantibody levels. Complete/partial remission was 88%/12% in pemphigus and 71%/29% in subepidermal blistering diseases. Overall relapse rate was 13% with an average follow-up of 22 months. In the AL332-MMP pt. the PAIA/rituximab treatment was stopped because of an oesophagus cancer considered as the paraneoplastic cause of the skin disease.Combined treatment with PAIA and rituximab showed rapid and long-lasting response, thereby allowing substantial reduction

2014 Journal of the European Academy of Dermatology and Venereology

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