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Pemphigus Foliaceus

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81. Elevated serum eosinophil cationic protein and transforming growth factor-α levels in a patient with pemphigus vegetans. (Abstract)

Elevated serum eosinophil cationic protein and transforming growth factor-α levels in a patient with pemphigus vegetans. Pemphigus vegetans (PVeg) is a rare variant of pemphigus, characterized by vegetating lesions mainly with antidesmoglein 3 antibodies. However, the pathomechanisms for PVeg is still unknown. We present a patient with PVeg mainly associated with antidesmocollin (Dsc)3 antibodies, who originally developed pemphigus foliaceus. Serum levels of eosinophil cationic protein

2018 Clinical & Experimental Dermatology

82. Coexistent Solid Malignancies in Pemphigus: A Population-Based Study. Full Text available with Trip Pro

Coexistent Solid Malignancies in Pemphigus: A Population-Based Study. The association of pemphigus vulgaris and pemphigus foliaceus with comorbid malignancies is yet to be firmly established.To estimate the association between pemphigus and a wide range of nonhematologic malignancies using one of the largest cohorts of patients with pemphigus to date.For this cross-sectional study, we used the computerized database of Clalit Health Services, the largest public health care provider organization (...) in Israel insuring 4.4 million individuals in the settings of general community clinics, primary care and referral centers, and ambulatory and hospitalized health care. The study included 1985 patients with pemphigus and 9874 control patients and was conducted from January 2004 to December 2014.The prevalence of 17 different solid malignancies was compared between patients diagnosed with pemphigus and age-, sex-, and ethnicity-matched control patients; χ2 and t tests were used for univariate analysis

2018 JAMA dermatology (Chicago, Ill.)

83. A prospective study comparing patients with early and late relapsing pemphigus treated with rituximab. (Abstract)

A prospective study comparing patients with early and late relapsing pemphigus treated with rituximab. Rituximab (RTX) is an effective therapy for patients with pemphigus; however, the therapy does not prevent relapse.To compare early relapsing patients (before 12 months) and late relapsing patients (after 24 months) following RTX therapy.In this prospective study, 19 patients were enrolled (14 with pemphigus vulgaris and 5 with pemphigus foliaceus). The baseline disease score, autoantibody (...) levels, and percentage of CD20+ cells of patients with pemphigus were measured. Patients received 1 cycle of RTX and were followed for 26 months.Among early relapsing patients (n = 5), the time to relapse was 6 to 11 months. Among late relapsing patients (n = 6), the time to relapse was 24 to 26 months. A significant difference was observed in the mean baseline anti-desmoglein 1 (DSG1) index between early relapsing (705.72) and late relapsing patients (210.4) (P = .0014). A significant negative

2018 Journal of American Academy of Dermatology

84. Pemphigus Foliaceus—Repeated Treatment With Rituximab 7 Years After Initial Response: A Case Report Full Text available with Trip Pro

Pemphigus Foliaceus—Repeated Treatment With Rituximab 7 Years After Initial Response: A Case Report Pemphigus foliaceus is an autoimmune skin disease mediated by autoantibodies directed against desmoglein-1 located in the upper epidermal layer. Rituximab, a monoclonal anit-CD20 antibody depleting b-cells, offers an effective treatment possibility for therapy-resistant pemphigus foliaceus. Here, we present the case of 55-year-old man who did not respond sufficiently to conventional treatment

2018 Frontiers in medicine

85. Paraneoplastic autoimmune multiorgan syndrome (PAMS): Beyond the single phenotype of paraneoplastic pemphigus. (Abstract)

including plakins, cadherins, alpha-2-macroglobulin like 1 (A2ML1), BP180, plakophilin-3, and several neuromuscular antigens. Originally described as paraneoplastic pemphigus in 1990 due to some of its clinical and immunologic similarities to classic pemphigus (pemphigus vulgaris and pemphigus foliaceus), PAMS is a multiorganopathy with several distinct features from these classic forms of pemphigus. Epidemiologically, PAMS is associated with underlying neoplasia and has a differing HLA-II allele (...) Paraneoplastic autoimmune multiorgan syndrome (PAMS): Beyond the single phenotype of paraneoplastic pemphigus. Paraneoplastic autoimmune multiorgan syndrome (PAMS) is characterized by a heterogenous group of signs and symptoms including severe desquamative stomatitis, a polymorphous cutaneous eruption, humoral immunity against plakin proteins, contribution of cell-mediated autoimmunity and commonly a progressive respiratory failure. Autoantibodies in PAMS target a wide array of antigens

2018 Autoimmunity reviews

86. A Study of PRN1008 in Patients With Pemphigus

patients with newly diagnosed or relapsing moderate to severe pemphigus (pemphigus vulgaris [PV] or pemphigus foliaceus [PF]) will be enrolled in the trial worldwide. The trial will last 68 weeks (approximately 17 months) for each participant. Patients will be randomized at Day 1, using a 1:1 ratio to receive PRN1008 or placebo twice per day, and by relapsing/newly diagnosed disease history (newly diagnosed defined as within 6 months of Screening). Study Design Go to Layout table for study information (...) to severe pemphigus, newly diagnosed or relapsing pemphigus vulgaris (PD) or pemphigus foliaceus (PF); positive anti-dsg1 or 3 autoantibody titer Laboratory clearance with adequate hematologic, hepatic, and renal function, body mass index (BMI) >17.5 Female patients who are of reproductive potential must agree for the duration of active treatment to use an effective means of contraception Able to provide written informed consent Exclusion Criteria: Previous use of a Bruton's tyrosine kinase (BTK

2018 Clinical Trials

87. Clinical and immunological features of pemphigus relapse. (Abstract)

prednisolone was tapered to around 0·1 mg kg-1 . In mucocutaneous pemphigus vulgaris (mcPV), the initial doses (mean ± SD) of prednisolone were significantly lower in patients with relapse (0·78 ± 0·24 mg kg-1 ) than in those without relapse (1·01 ± 0·01 mg kg-1 ). At relapse, mcPV shifted to mucosal dominant PV (mPV; 40%), pemphigus foliaceus (PF) (20%) or 'other' (20%). In contrast, relapsing mPV and PF had the same clinical phenotypes as the initial phenotypes. Patients with both anti-desmoglein (Dsg)1 (...) Clinical and immunological features of pemphigus relapse. More than half of patients with pemphigus experience relapse during the disease course. The risk factors and clinical and immunological characteristics of relapse remain largely unclear.To elucidate the risk factors and clinical features of pemphigus relapse.We carried out a retrospective review of the clinical records of 42 cases of pemphigus at a single centre.Sixty-two per cent of patients experienced relapse, usually when oral

2018 British Journal of Dermatology

88. Pemphigus Foliaceus

Pemphigus Foliaceus Pemphigus Foliaceus Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Pemphigus Foliaceus Pemphigus Foliaceus Aka (...) : Pemphigus Foliaceus , Superficial Pemphigus , Pemphigus erythematosus , Senear-Usher Syndrome , Fogo selvagem , Pemphigus herpetiformis From Related Chapters II. Definition Less common, milder and superficial form of III. Signs Onset on skin of face in butterfly distribution May also involve scalp, chest or upper back Rarely involves absent IV. Variants Pemphigus erythematosus (Senear-Usher Syndrome) Localized variant of Pemphigus Foliaceus Associated with positive Fogo selvagem Variant of Pemphigus

2015 FP Notebook

89. Brazilian pemphigus foliaceus anti-desmoglein 1 autoantibodies cross-react with sand fly salivary LJM11 antigen Full Text available with Trip Pro

Brazilian pemphigus foliaceus anti-desmoglein 1 autoantibodies cross-react with sand fly salivary LJM11 antigen The environmental factors that contribute to the development of autoimmune diseases are largely unknown. Endemic pemphigus foliaceus in humans, known as Fogo Selvagem (FS) in Brazil, is mediated by pathogenic IgG4 autoantibodies against desmoglein 1 (Dsg1). Clusters of FS overlap with those of leishmaniasis, a disease transmitted by sand fly (Lutzomyia longipalpis) bites

2012 Journal of immunology (Baltimore, Md. : 1950)

90. Endemic pemphigus foliaceus: towards understanding autoimmune mechanisms of disease development. Full Text available with Trip Pro

Endemic pemphigus foliaceus: towards understanding autoimmune mechanisms of disease development. Fogo selvagem is an endemic form of pemphigus foliaceus (EPF) found in Brazil. Environmental and genetic factors are thought to contribute to the disease, which is associated with pathogenic IgG4 autoantibodies against the desmosomal cadherin desmoglein 1. In this issue, as an additional framework to understand autoimmune mechanisms in EPF, Flores et al. have investigated whether fogo selvagem

2012 Journal of Investigative Dermatology

91. Profile of Trypanosoma cruzi Reactivity in a Population at High Risk for Endemic Pemphigus Foliaceus (Fogo Selvagem). Full Text available with Trip Pro

Profile of Trypanosoma cruzi Reactivity in a Population at High Risk for Endemic Pemphigus Foliaceus (Fogo Selvagem). Fogo selvagem (FS) is an autoimmune bullous disease with pathogenic IgG autoantibodies recognizing desmoglein 1 (Dsg1), a desmosomal glycoprotein. In certain settlements of Brazil, a high prevalence of FS (3%) is reported, suggesting environmental factors as triggers of the autoimmune response. Healthy individuals from endemic areas recognize nonpathogenic epitopes of Dsg1

2012 American Journal of Tropical Medicine & Hygiene

92. Clearance Efficacy of Autoantibodies in Double Filtration Plasmapheresis for Pemphigus Foliaceus. Full Text available with Trip Pro

Clearance Efficacy of Autoantibodies in Double Filtration Plasmapheresis for Pemphigus Foliaceus. 22948906 2013 09 24 2015 11 19 1651-2057 93 2 2013 Mar 27 Acta dermato-venereologica Acta Derm. Venereol. Clearance efficacy of autoantibodies in double filtration plasmapheresis for pemphigus foliaceus. 181-2 10.2340/00015555-1444 Kasuya Akira A Moriki Mutsumi M Tatsuno Kazuki K Hirakawa Satoshi S Tokura Yoshiki Y eng Case Reports Letter Sweden Acta Derm Venereol 0370310 0001-5555 0 Autoantibodies (...) 0 Biomarkers 0 DSG1 protein, human 0 Desmoglein 1 0 Immunoglobulin A 0 Immunoglobulin G 0 Immunoglobulin M IM Aged, 80 and over Autoantibodies blood Biomarkers blood Desmoglein 1 immunology Humans Immunoglobulin A blood Immunoglobulin G blood Immunoglobulin M blood Male Middle Aged Pemphigus blood immunology therapy Plasmapheresis adverse effects methods Treatment Outcome 2012 9 6 6 0 2012 9 6 6 0 2013 9 26 6 0 ppublish 22948906 10.2340/00015555-1444

2012 Acta Dermato-Venereologica

93. IgG Autoantibody Response against Keratinocyte Cadherins in Endemic Pemphigus Foliaceus (Fogo Selvagem). Full Text available with Trip Pro

IgG Autoantibody Response against Keratinocyte Cadherins in Endemic Pemphigus Foliaceus (Fogo Selvagem). It is well established that autoantibodies against desmoglein 3 and desmoglein 1 (Dsg1) are relevant in the pathogenesis of pemphigus vulgaris and pemphigus foliaceus, including its endemic form fogo selvagem (FS). Isolated reports have shown that in certain patients with these diseases, autoantibodies against other desmosomal cadherins and E-cadherin may also be present. The goal

2012 Journal of Investigative Dermatology

94. Ultrastructure of acantholysis in pemphigus foliaceus reexamined from the current perspective. Full Text available with Trip Pro

Ultrastructure of acantholysis in pemphigus foliaceus reexamined from the current perspective. Pemphigus foliaceus (PF) is a chronic cutaneous autoimmune blistering disease that is characterized by superficial blistering of the skin, and according to the current perspective is caused by autoantibodies directed against desmoglein (Dsg) 1.To examine early acantholysis in the skin of patients with PF at an ultrastructural level.Two Nikolsky-negative (N-), five Nikolsky-positive (N+) and two

2012 British Journal of Dermatology

95. Pemphigus Foliaceus Full Text available with Trip Pro

Pemphigus Foliaceus 19982227 2010 06 24 2010 06 24 0035-9157 15 Dermatol Sect 1922 Proceedings of the Royal Society of Medicine Proc. R. Soc. Med. Pemphigus Foliaceus. 20 Gray A M AM eng Journal Article England Proc R Soc Med 7505890 0035-9157 2009 12 9 6 0 1922 1 1 0 0 1922 1 1 0 1 ppublish 19982227 PMC2103260

1922 Proceedings of the Royal Society of Medicine

96. Pemphigus Foliaceus Full Text available with Trip Pro

Pemphigus Foliaceus 19991532 2010 06 24 2010 06 24 0035-9157 31 8 1938 Jun Proceedings of the Royal Society of Medicine Proc. R. Soc. Med. Pemphigus Foliaceus. 871 Gray A M AM eng Journal Article England Proc R Soc Med 7505890 0035-9157 2009 12 9 6 0 1938 6 1 0 0 1938 6 1 0 1 ppublish 19991532 PMC2076800

1938 Proceedings of the Royal Society of Medicine

97. Pemphigus Foliaceus Full Text available with Trip Pro

Pemphigus Foliaceus 19992758 2010 06 24 2010 06 24 0035-9157 37 3 1944 Jan Proceedings of the Royal Society of Medicine Proc. R. Soc. Med. Pemphigus Foliaceus. 73-4 Wigley J E JE eng Journal Article England Proc R Soc Med 7505890 0035-9157 2009 12 9 6 0 1944 1 1 0 0 1944 1 1 0 1 ppublish 19992758 PMC2180884

1944 Proceedings of the Royal Society of Medicine

98. Pemphigus Foliaceus Full Text available with Trip Pro

Pemphigus Foliaceus 19992775 2010 06 24 2010 06 24 0035-9157 37 4 1944 Feb Proceedings of the Royal Society of Medicine Proc. R. Soc. Med. Pemphigus Foliaceus. 153 Forman L L eng Journal Article England Proc R Soc Med 7505890 0035-9157 2009 12 9 6 0 1944 2 1 0 0 1944 2 1 0 1 ppublish 19992775 PMC2180915

1944 Proceedings of the Royal Society of Medicine

99. T and B lymphocytes in South American pemphigus foliaceus. Full Text available with Trip Pro

T and B lymphocytes in South American pemphigus foliaceus. T and B lymphocytes were quantified in peripheral blood of thirty patients with South American pemphigus foliaceus according to their ability to form rosettes with sheep erythrocytes (E) or sheep erythrocytes sensitized with antibody an complement (EAC). When compared with the counts obtained from thirty normal subjects, a decrease was found in the total T-lymphocyte count (32-33+/-7-7 versus 46-7+/-8-7) and in the T functional (...) American pemphigus foliaceus may reflect an impaired cellular immunity.

1976 Clinical and experimental immunology

100. Pemphigus foliaceus induced by penicillamine. Full Text available with Trip Pro

Pemphigus foliaceus induced by penicillamine. 1009359 1977 03 15 2018 11 13 0007-1447 2 6049 1976 Dec 11 British medical journal Br Med J Pemphigus foliaceus induced by penicillamine. 1423-4 Marsden R A RA Vanhegan R I RI Walshe M M Hill H H Mowat A G AG eng Case Reports Journal Article England Br Med J 0372673 0007-1447 GNN1DV99GX Penicillamine AIM IM Aged Arthritis, Rheumatoid drug therapy Female Humans Male Middle Aged Pemphigus chemically induced Penicillamine adverse effects 1976 12 11

1976 British medical journal

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