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Pemphigus Foliaceus

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61. Clinical and immunological profile of umbilical involvement in pemphigus vulgaris and pemphigus foliaceus. (Abstract)

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

62. 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

63. 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

64. 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

65. 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

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

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

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

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

68. Autoimmune reactivity against precursor form of desmoglein 1 in healthy Tunisians in the area of endemic pemphigus foliaceus. Full Text available with Trip Pro

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

2013 Journal of dermatological science

69. Pemphigus Foliaceus Associated with Anti-NXP2 Autoantibody-positive Dermatomyositis. Full Text available with Trip Pro

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

2013 Acta Dermato-Venereologica

70. Endemic pemphigus foliaceus in the Peruvian Amazon. (Abstract)

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

71. Biomarkers of oxidative stress in epidermis of Tunisian pemphigus foliaceus patients. (Abstract)

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

72. Investigation of the Predisposing Factor of Pemphigus and its clinical subtype through a Genome-wide association and next generation sequence analysis. (Abstract)

Investigation of the Predisposing Factor of Pemphigus and its clinical subtype through a Genome-wide association and next generation sequence analysis. Pemphigus is an autoimmune blistering disease with pemphigus vulgaris (PV) and foliaceus (PF) as the two major histological subtypes. Associations with HLA molecules have been suggested, but specific HLA risk variants as well as non-HLA risk variants remain to be discovered.We performed a two-stage genome-wide association study in the Chinese (...) understanding of the genetic basis of pemphigus susceptibility and may offer opportunities for risk prediction and preventive treatment for pemphigus, in particular for PV.© 2018 European Academy of Dermatology and Venereology.

2018 Journal of the European Academy of Dermatology and Venereology

73. Large International Validation of ABSIS and PDAI Pemphigus Severity Scores. Full Text available with Trip Pro

in a large international study. Consecutive patients with newly diagnosed pemphigus were enrolled in 31 centers. Severity scores were recorded during a 24-month period by the same two blinded investigators. Serum was collected at each visit for ELISA measurement of anti-desmoglein antibodies. The intraclass correlation coefficient (ICC) and Spearman rank correlation coefficient were calculated. A total of 116 patients with pemphigus vulgaris (n = 84) or pemphigus foliaceus (n = 32) were included (...) Large International Validation of ABSIS and PDAI Pemphigus Severity Scores. The Pemphigus Disease Area Index (PDAI) and Autoimmune Bullous Skin Disorder Intensity-Score (ABSIS) scores have been proposed to provide an objective measure of pemphigus activity. These scores have been evaluated only on already treated patients mainly with mild to moderate activity. The objective was to assess the interrater reliability of ABSIS and PDAI scores and their correlation with other severity markers

2018 Journal of Investigative Dermatology

74. Subtype-Specific Inherited Predisposition to Pemphigus in Chinese Population. (Abstract)

Subtype-Specific Inherited Predisposition to Pemphigus in Chinese Population. Pemphigus is a group of rare life-threatening mucocutaneous autoimmune diseases, presenting mainly as two subtypes: pemphigus vulgaris (PV) and pemphigus foliaceus (PF). Inherited predispositions to pemphigus have long been speculated but they remain poorly understood.To identify common and specific nongenetic and genetic factors associated with pemphigus and its subtypes in the Chinese population.A genome-wide (...) association study (GWAS) was performed in 496 unrelated patients with pemphigus (including 365 with PV and 104 with PF) and 1105 controls without pemphigus.A sex preference was observed only in PV (57·5% female) and not in PF (47·1% female). For male patients only, the mean age at diagnosis was significantly lower for PV than for PF (P < 0·001). The strongest associated single-nucleotide polymorphisms are in the human leucocyte antigen (HLA) region: rs70993900 (PV; P = 1·5 × 10-45 ) and rs9469220 (PF; P

2018 British Journal of Dermatology

75. The Usefulness of Indirect Immunofluorescence in Pemphigus and the Natural History of Patients With Initial False-Positive Results: A Retrospective Cohort Study Full Text available with Trip Pro

diagnosed with pemphigus vulgaris (PV) and 29 patients with pemphigus foliaceus (PF). The sensitivity of this immunoassay was significantly higher for the diagnosis of PV (87.4%; 95% CI, 81.5-91.9%) as compared to PF (69.0%; 95% CI, 49.2-84.7%; P = 0.018). The specificity for the diagnosis of pemphigus was 93.5% (95% CI, 91.1-95.4%). Patients with false-positive results (n = 37) were followed for a median duration of 5.3 years contributing 280.8 person-years. Thirty patients (81.1%) were eventually (...) The Usefulness of Indirect Immunofluorescence in Pemphigus and the Natural History of Patients With Initial False-Positive Results: A Retrospective Cohort Study The specificity and the predictive values of indirect immunofluorescence (IIF) in real-life settings is yet to be firmly established. The natural history of patients with false-positive results has not been sufficiently elucidated. The primary aim of the current study is to evaluate the diagnostic value of IIF analysis on monkey

2018 Frontiers in medicine

76. Usefulness of miRNA-338-3p in the diagnosis of pemphigus and its correlation with disease severity Full Text available with Trip Pro

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

2018 PeerJ

77. Keratins Regulate p38MAPK-Dependent Desmoglein Binding Properties in Pemphigus Full Text available with Trip Pro

Keratins Regulate p38MAPK-Dependent Desmoglein Binding Properties in Pemphigus Keratins are crucial for the anchorage of desmosomes. Severe alterations of keratin organization and detachment of filaments from the desmosomal plaque occur in the autoimmune dermatoses pemphigus vulgaris and pemphigus foliaceus (PF), which are mainly caused by autoantibodies against desmoglein (Dsg) 1 and 3. Keratin alterations are a structural hallmark in pemphigus pathogenesis and correlate with loss (...) of intercellular adhesion. However, the significance for autoantibody-induced loss of intercellular adhesion is largely unknown. In wild-type (wt) murine keratinocytes, pemphigus autoantibodies induced keratin filament retraction. Under the same conditions, we used murine keratinocytes lacking all keratin filaments (KtyII k.o.) as a model system to dissect the role of keratins in pemphigus. KtyII k.o. cells show compromised intercellular adhesion without antibody (Ab) treatment, which was not impaired further

2018 Frontiers in immunology

78. The Significance of Scalp Involvement in Pemphigus: A Literature Review Full Text available with Trip Pro

with pemphigus. Follicular acantholysis is a characteristic histopathological feature of pemphigus lesions localized on the scalp. Trichoscopy may serve as a supplementary method in the diagnosis of pemphigus. This review summarizes the most recent data concerning scalp involvement in pemphigus vulgaris and pemphigus foliaceus. A systematic literature search was conducted in three medical databases: PubMed, Embase, and Web of Science. The analysis included literature data about desmoglein distribution (...) The Significance of Scalp Involvement in Pemphigus: A Literature Review Scalp is a unique location for pemphigus because of the abundance of desmogleins localized in hair follicles. Scalp involvement is observed in up to 60% of patients in the course of pemphigus. The lesions may occasionally lead to alopecia. Unforced removal of anagen hairs in a pull test is a sign of high disease activity. Direct immunofluorescence of plucked hair bulbs is considered a reliable diagnostic method in patients

2018 BioMed research international

79. Retrospective analysis of a single-center clinical experience toward development of curative treatment of 123 pemphigus patients with a long-term follow-up: efficacy and safety of the multidrug protocol combining intravenous immunoglobulin with the cytoto Full Text available with Trip Pro

saving, the high dose and prolonged courses required for disease control are associated with significant adverse effects, including death. Although introduction of rituximab (RTX) provided for a favorable outcome, the high relapse rate, that is, up to 80%, precludes successful use of RTX as a monotherapy. Intravenous immunoglobulin (IVIg) is being increasingly utilized as off-label therapy for a variety of autoimmune and inflammatory diseases, including PV and pemphigus foliaceus (PF).The goal (...) Retrospective analysis of a single-center clinical experience toward development of curative treatment of 123 pemphigus patients with a long-term follow-up: efficacy and safety of the multidrug protocol combining intravenous immunoglobulin with the cytoto Pemphigus vulgaris (PV) is a life-long IgG autoantibody-mediated blistering disease affecting the mucosal surfaces lined by the stratified epithelium (oral, nasal, genital) and sometimes also the skin. While corticosteroid treatment is life

2018 International Journal of Dermatology

80. Rituximab and Low Dose Oral Immune Modulating Treatment to Maintain a Sustained Response in Severe Pemphigus Patients. (Abstract)

Rituximab and Low Dose Oral Immune Modulating Treatment to Maintain a Sustained Response in Severe Pemphigus Patients. This pilot retrospective study examined the role of continuous low-dose maintenance immunomodulatory treatment (IMT) as an adjunct to rituximab (RTX) rescue therapy in severe pemphigus vulgaris (PV) and pemphigus foliaceus (PF) after a complete response (CR) to RTX was achieved.Ten severe pemphigus patients who received RTX rescue therapy were evaluated after achieving CR (...) propose an alternative treatment strategy utilizing RTX as a rescue agent in combination with long-term LRMD as a means to maintain a sustained long-term CR post-RTX therapy in severe pemphigus patients. This strategy could prevent disease flares and the need for additional RTX cycles and higher dosages of immunomodulatory therapies.

2018 Journal of Dermatological Treatment

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