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Pemphigus Foliaceus

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61. Pemphigus Foliaceus (Overview)

Pemphigus Foliaceus (Overview) Pemphigus Foliaceus: 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=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTA2NDAxOS1vdmVydmlldw== processing > Pemphigus Foliaceus Updated: Apr 03 (...) , 2018 Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD Share Email Print Feedback Close Sections Sections Pemphigus Foliaceus Overview Background Pemphigus foliaceus (PF) is generally a benign variety of pemphigus. It is an autoimmune skin disorder characterized by the loss of intercellular adhesion of keratinocytes in the upper parts of the epidermis (acantholysis), resulting in the formation of superficial blisters. It is typified by clinical involvement of healthy-appearing

2014 eMedicine.com

62. Pemphigus Foliaceus (Treatment)

Pemphigus Foliaceus (Treatment) Pemphigus Foliaceus Treatment & Management: Medical Care 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=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTA2NDAxOS10cmVhdG1lbnQ= processing > Pemphigus Foliaceus Treatment (...) & Management Updated: Apr 03, 2018 Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD Share Email Print Feedback Close Sections Sections Pemphigus Foliaceus Treatment Medical Care Present information is probably inadequate to ascertain the optimal therapy for pemphigus foliaceus (PF), including the optimal glucocorticoid dose, the role of adjuvant immunosuppressive medications, and long-term adverse events to improve the risk-to-benefit ratio. [ ] Therapy for pemphigus foliaceus is usually

2014 eMedicine.com

63. Pemphigus Foliaceus (Follow-up)

Pemphigus Foliaceus (Follow-up) Pemphigus Foliaceus Treatment & Management: Medical Care 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=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTA2NDAxOS10cmVhdG1lbnQ= processing > Pemphigus Foliaceus Treatment (...) & Management Updated: Apr 03, 2018 Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD Share Email Print Feedback Close Sections Sections Pemphigus Foliaceus Treatment Medical Care Present information is probably inadequate to ascertain the optimal therapy for pemphigus foliaceus (PF), including the optimal glucocorticoid dose, the role of adjuvant immunosuppressive medications, and long-term adverse events to improve the risk-to-benefit ratio. [ ] Therapy for pemphigus foliaceus is usually

2014 eMedicine.com

64. Pemphigus Foliaceus (Diagnosis)

Pemphigus Foliaceus (Diagnosis) Pemphigus Foliaceus: 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=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTA2NDAxOS1vdmVydmlldw== processing > Pemphigus Foliaceus Updated: Apr 03 (...) , 2018 Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD Share Email Print Feedback Close Sections Sections Pemphigus Foliaceus Overview Background Pemphigus foliaceus (PF) is generally a benign variety of pemphigus. It is an autoimmune skin disorder characterized by the loss of intercellular adhesion of keratinocytes in the upper parts of the epidermis (acantholysis), resulting in the formation of superficial blisters. It is typified by clinical involvement of healthy-appearing

2014 eMedicine.com

65. Direct immunofluorescence of the outer root sheath in anagen and telogen hair in pemphigus vulgaris and pemphigus foliaceus. (PubMed)

Direct immunofluorescence of the outer root sheath in anagen and telogen hair in pemphigus vulgaris and pemphigus foliaceus. Direct immunofluorescence of peri-lesional skin is the gold standard in the diagnosis of pemphigus. A specific immunofluorescence pattern may also be demonstrated in the outer root sheath of anagen and telogen hair. We demonstrated an intercellular reticular deposition of immunoglobulin G in the outer root sheath of plucked anagen and telogen hair in all pemphigus (...) vulgaris patients with active disease and for the first time in all patients with active pemphigus foliaceus. Moreover, we demonstrated for the first time that plucked hair samples may be kept at -20°C for at least 2 weeks before immunofluorescent staining and analysis. © 2013 The Authors. Australasian Journal of Dermatology © 2013 The Australasian College of Dermatologists.

2013 Australasian Journal of Dermatology

66. Prognostic factors in pemphigus vulgaris and pemphigus foliaceus. (PubMed)

Prognostic factors in pemphigus vulgaris and pemphigus foliaceus. Pemphigus typically has a chronic course, although there is great variability in disease duration (DD) and time taken to disease remission (DR) between individuals with the disease. The reasons for this are unclear.To explore the prognostic influence of epidemiological, clinical, immunological and genetic factors on disease course and remission in pemphigus vulgaris (PV) and pemphigus foliaceus (PF).This was a retrospective study (...) anti-Dsg 3 levels at baseline were associated with a longer total DD (P = 0.03).Ethnic group, age at presentation, initial intercellular antibody titre and initial Dsg 3 antibody levels all had a significant impact on prognosis of pemphigus.© 2013 British Association of Dermatologists.

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2013 British Journal of Dermatology

67. Pemphigus Foliaceus Masquerading as IgA Pemphigus and Responding to Dapsone (PubMed)

Pemphigus Foliaceus Masquerading as IgA Pemphigus and Responding to Dapsone A 14-year-old male presented with seven years history of recurrent episodes of fluid filled, itchy and eroded lesions over the body not responding to oral corticosteroids and azathioprine. Dermatological examination revealed crusted plaques and erosions in a seborrheic distribution. Histopathology of skin lesions and direct immunofluorescence were characteristic of pemphigus foliaceus. He was treated with dexamethasone (...) pulse therapy with inadequate response. However, relapsing skin lesions revealed a circinate arrangement with a predilection to trunk and flexures. In view of clinical features suggestive of IgA pemphigus, he was started on dapsone, to which he responded dramatically in four weeks. However, repeat biopsy continued to reveal features of pemphigus foliaceus and ELISA for anti-desmoglein 1 antibodies was positive.

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2012 Indian journal of dermatology

68. Clinical and immunological profile of umbilical involvement in pemphigus vulgaris and pemphigus foliaceus. (PubMed)

Clinical and immunological profile of umbilical involvement in pemphigus vulgaris and pemphigus foliaceus. Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune vesicobullous disorders with IgG autoantibodies directed against desmoglein (Dsg)1 and 3, which lead to intraepidermal acantholysis.To characterize the clinical and immunological profile of patients with PF or PV with umbilical involvement.In total, 10 patients (7 women, 3 men; age range 24-70 years, disease duration 3-16 (...) in the other two. Indirect immunofluorescence with IgG conjugate showing the typical pemphigus pattern was positive in all 10 patients, with titres varying from 1 : 160 to 1 : 2560. ELISA with recombinant Dsg1 gave scores of 24-266 in PF and 0-270 in PV. Reactivity to recombinant Dsg3 was positive in all five patients with PV (ELISA 22-98) and was negative in all PF sera.All 10 patients with pemphigus with umbilical presentation had the clinical and immunopathological features of either PF or PV

2012 Clinical & Experimental Dermatology

69. Calculation of cut-off values based on the ABSIS and PDAI pemphigus scoring systems for defining moderate, significant and extensive types of pemphigus. (PubMed)

and 75th percentiles of the ABSIS and PDAI scores. The median ABSIS, PDAI, PGA and DLQI scores of the three severity subgroups were compared in order to validate these subgroups.Ninety-six patients with pemphigus vulgaris (n = 77) or pemphigus foliaceus (n = 19) were included. The median PDAI activity and ABSIS total scores were 27·5 (range 3-84) and 34·8 points (range 0·5-90·5), respectively. The respective cut-off values corresponding to the first and third quartiles of the scores were 15 and 45 (...) Calculation of cut-off values based on the ABSIS and PDAI pemphigus scoring systems for defining moderate, significant and extensive types of pemphigus. Two pemphigus severity scores, Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and Pemphigus Disease Area Index (PDAI), have been proposed to provide an objective measure of disease activity. However, the use of these scores in clinical practice is limited by the absence of cut-off values that allow differentiation between moderate

2016 British Journal of Dermatology

70. Inter-rater reliability of the BIOCHIP indirect immunofluorescence dermatology mosaic in bullous pemphigoid and pemphigus patients. (PubMed)

Inter-rater reliability of the BIOCHIP indirect immunofluorescence dermatology mosaic in bullous pemphigoid and pemphigus patients. The BIOCHIP (Dermatology Mosaic 7; EUROIMMUN, Lubeck, Germany) is a novel multiplex indirect immunofluorescence (IIF) technique used in the serological diagnosis of bullous pemphigoid (BP) and pemphigus.To validate the accuracy and inter-rater reliability (IRR) of the BIOCHIP in the diagnosis of BP, pemphigus foliaceus (PF) and pemphigus vulgaris (PV).Sera from

2019 Journal of the European Academy of Dermatology and Venereology

71. Autoreactive B cell differentiation in diffuse ectopic lymphoid-like structures of inflamed pemphigus lesions. (PubMed)

Autoreactive B cell differentiation in diffuse ectopic lymphoid-like structures of inflamed pemphigus lesions. Pemphigus is an organ-specific autoimmune disease targeting skin and/or mucous membranes. Our previous study showed that infiltrating lymphocytes in pemphigus vulgaris (PV) lesions produce anti-desmoglein (Dsg) 1/3 antibodies after in vitro culture. In this study, we found diffuse ectopic lymphoid-like structures (ELSs) commonly present in lesions of both PV and pemphigus foliaceus (PF (...) ). Notably, pemphigus lesions contained centroblasts, plasmablasts and plasma cells, which recapitulated the different stages of B cell differentiation. Elevated mRNA expression levels of the differentiation-related transcription factors BLIMP-1, IRF4 and BCL-6 were observed in pemphigus lesions. Moreover, B cell receptor (BCR) repertoire analysis revealed the clonal expansion of lesional B cells. Lesional B cells might recirculate among lesions, lymph nodes and peripheral blood. Increased mRNA

2019 Journal of Investigative Dermatology

72. Autoimmune reactivity against precursor form of desmoglein 1 in healthy Tunisians in the area of endemic pemphigus foliaceus. (PubMed)

Autoimmune reactivity against precursor form of desmoglein 1 in healthy Tunisians in the area of endemic pemphigus foliaceus. Desmoglein 1 (Dsg1), the pemphigus foliaceus (PF) antigen, is produced as a precursor (preDsg1) and is transported to the cell surface as the mature form (matDsg1). Recent studies show that B cells from North American individuals without pemphigus can potentially produce anti-preDsg1 IgG antibodies, but ELISA screening of large numbers of normal people in North America

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2013 Journal of dermatological science

73. Pemphigus Foliaceus Associated with Anti-NXP2 Autoantibody-positive Dermatomyositis. (PubMed)

Pemphigus Foliaceus Associated with Anti-NXP2 Autoantibody-positive Dermatomyositis. 24217940 2015 03 09 2015 11 19 1651-2057 94 4 2014 Jul Acta dermato-venereologica Acta Derm. Venereol. Pemphigus foliaceus associated with anti-NXP2 autoantibody-positive dermatomyositis. 478-9 10.2340/00015555-1756 Fujimoto Noriki N Department of Dermatology, Shiga University of Medical Science, Setatsukinowa, Otsu, Shiga 520-2192, Japan, noriki@belle.shiga-med.ac.jp. Takayama Satoru S Hamaguchi Yasuhito Y (...) -Binding Proteins immunology Dermatomyositis blood drug therapy enzymology immunology Desmoglein 1 immunology Female Humans Pemphigus blood diagnosis drug therapy immunology Treatment Outcome 2013 11 13 6 0 2013 11 13 6 0 2015 3 10 6 0 ppublish 24217940 10.2340/00015555-1756

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2013 Acta Dermato-Venereologica

74. Endemic pemphigus foliaceus in the Peruvian Amazon. (PubMed)

Endemic pemphigus foliaceus in the Peruvian Amazon. Endemic pemphigus foliaceus (EPF) is an organ-specific blistering disease of the epidermis characterized by the presence of IgG autoantibodies, specifically desmoglein (Dsg)1. This condition has been reported particularly in Brazil, Colombia, Tunisia and Peru.To characterize the humoral response against Dsg1 and Dsg3 autoantibodies of patients with EPF from the Peruvian Amazon region.Blood samples were collected from 16 patients

2013 Clinical & Experimental Dermatology

75. Biomarkers of oxidative stress in epidermis of Tunisian pemphigus foliaceus patients. (PubMed)

Biomarkers of oxidative stress in epidermis of Tunisian pemphigus foliaceus patients. Reactive oxygen species play a key role in the development of many dermatological disorders.The purpose of this study is to examine the lipid peroxidation, protein oxidation and antioxidative profile in Tunisian pemphigus foliaceus (PF) patients.Malondialdehyde (MDA), conjugated dienes (CD), protein thiol levels, catalase (CAT) and superoxide dismutase (SOD) activities were evaluated in skin biopsies of 13

2013 Journal of the European Academy of Dermatology and Venereology

76. Evidence-based treatments for pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid: a systematic review.

Evidence-based treatments for pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid: a systematic review. Pemphigus, bullous pemphigoid, and epidermolysis bullosa acquisita are autoimmune diseases of skin associated with considerable morbidity and sometimes mortality. There is no cure for these diseases.To summarize evidence-based treatments for these diseases by performing a systematic review.The research protocol included the following steps: identification of databases (...) be found. After the second-stage screening, 12 randomized controlled trials were analyzed, which included patients with pemphigus vulgaris or pemphigus vulgaris and pemphigus foliaceus, and 7 which included patients with bullous pemphigoid.Number of high-quality randomized controlled trials conducted on pemphigus and bullous pemphigoid is small. Oral corticosteroid along with a steroid-sparing agent appears to be the most effective treatment for pemphigus. Azathioprine may be most effective

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2011 Indian journal of dermatology, venereology and leprology

77. A systematic review of randomized controlled trials for pemphigus vulgaris and pemphigus foliaceus. (PubMed)

A systematic review of randomized controlled trials for pemphigus vulgaris and pemphigus foliaceus. A range of interventions has been described for the treatment of pemphigus; however, the optimal therapeutic strategy has not been established.We sought to evaluate the safety and efficacy of interventions for pemphigus vulgaris and pemphigus foliaceus.We undertook a systematic review and meta-analysis according to the methodology of the Cochrane Collaboration. We selected randomized controlled (...) trials including participants with the diagnosis of pemphigus vulgaris or pemphigus foliaceus confirmed with clinical, histopathological, and immunofluorescence criteria. All interventions were considered. Primary outcomes studied were remission and mortality. Secondary outcomes included disease control, relapse, pemphigus severity score, time to disease control, cumulative glucocorticoid dose, serum antibody titers, adverse events, and quality of life.Eleven studies with a total of 404 participants

2011 Journal of the American Academy of Dermatology

78. Vulvo-cervico-vaginal manifestations and evaluation of Papanicolaou smears in pemphigus vulgaris and pemphigus foliaceus. (PubMed)

Vulvo-cervico-vaginal manifestations and evaluation of Papanicolaou smears in pemphigus vulgaris and pemphigus foliaceus. Vulvo-cervico-vaginal involvement has rarely been reported in pemphigus vulgaris (PV) and has not been reported in pemphigus foliaceus (PF).We sought to evaluate genital lesions and Papanicolaou (Pap) smears in female patients with PV and PF.This prospective study includes all consecutive cases of female patients with PV and PF seen from May 2009 to February 2010 (...) . Gynecologic examination was performed and Pap smears were collected for cytologic analysis from each patient.A total of 56 patients were given a diagnosis of pemphigus (41 PV and 15 PF). Genital involvement was observed in 9 patients with PV (22%) and the vulva was the most common genital site of involvement. Of these 9 patients, 8 presented with active skin/mucous lesions. Four of 15 patients with PF had genital lesions and vulva was the exclusive site of involvement. Three of 4 patients with PF

2011 Journal of American Academy of Dermatology

79. Analysis of the reactivity of indirect immunofluorescence in patients with pemphigus foliaceus and pemphigus vulgaris using rat bladder epithelium as a substrate (PubMed)

Analysis of the reactivity of indirect immunofluorescence in patients with pemphigus foliaceus and pemphigus vulgaris using rat bladder epithelium as a substrate To evaluate the reactivity of indirect immunofluorescence using rat bladder epithelium as a substrate in patients with pemphigus foliaceus and pemphigus vulgaris from the Department of Dermatology, University of São Paulo Medical School, Brazil.Thirty-two patients (8 male and 24 female) from the Department of Dermatology, University (...) of São Paulo Medical School, were selected. Three had mucosal pemphigus vulgaris, 20 had mucocutaneous pemphigus vulgaris, and 9 had pemphigus foliaceus. Patients' sera were tested by indirect immunofluorescence performed on human foreskin and rat bladder epithelium and by ELISA assays utilizing baculovirus-expressed recombinant desmoglein 3 and desmoglein 1.No patients with mucosal pemphigus vulgaris, 5 of 20 patients with mucocutaneous pemphigus vulgaris (25%) and 4 of 9 patients with pemphigus

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2011 Clinics

80. Usefulness of miRNA-338-3p in the diagnosis of pemphigus and its correlation with disease severity (PubMed)

as diagnostic biomarkers for some autoimmune diseases. Previously, miR-338-3p has been proven significantly up-regulated in pemphigus patients.Pemphigus patients (including pemphigus vulgaris and pemphigus foliaceus) with active lesions and with remission, patients diagnosed as bullous pemphigoid and healthy volunteers were recruited, and miR-338-3p expression level was measured using reverse transcriptase-quantitative polymerase chain reaction (RT-qPCR). Active pemphigus patients accepting treatment were (...) Usefulness of miRNA-338-3p in the diagnosis of pemphigus and its correlation with disease severity Pemphigus is a common life-threatening, autoimmune bullous disease effecting both cutaneous and mucous membranes. Previous diagnosis of pemphigus is based on clinical presentations, histopathology, immunofluorescence and enzyme-linked immunosorbent assay. Furthermore, no laboratory parameters could be used to indicate disease severity. MicroRNAs are endogenous small RNAs, which could be used

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2018 PeerJ

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