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Background: Prospective studies identifying immunological parameters that can predict clinical relapse in pemphigus are scarce.
Objective: To periodically assess immunological parameters in patients with pemphigus vulgaris and foliaceous in remission to understand immunological events preceding clinical relapse.
Methods: A total of 105 patients were included. Baseline assessment included direct immunofluorescence (DIF), serum IgG against desmoglein (Dsg) 1, IgG, IgG1, and IgG4 against Dsg 3, IgG against the extracellular domains 1 and 2 of Dsg 3, IgG against muscarinic (M3)-AchR, and peripheral CD19+CD27+ memory B cells/plasma cells, repeated every 3 months for up to 12 months or until clinical relapse. DIF was repeated at month 12 and on relapse.
Results: About 29 of 105 patients (28%) experienced a relapse. Longer duration of clinical remission, presence of pruritus and positive anti-Dsg1 at baseline correlated with higher relapse rates. Compared with the visit immediately preceding relapse, a significantly increased number of patients with positive anti-Dsg1 (38% vs 31.1%, = .01), anti-Dsg3 (51.7% vs 41.4%, = .01) and IgG positivity by DIF (85.7% vs 25%, < .001) was observed at the time of relapse.
Conclusion: Regular monitoring of anti-Dsg 1 and anti-Dsg 3 serum levels and DIF positivity during the course of the disease in remission may predict relapse.
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http://dx.doi.org/10.1016/j.jdin.2024.12.004 | DOI Listing |
Int J Mol Sci
April 2025
Department of Allergy & Immunology, Huashan Hospital, Fudan University, Shanghai 200437, China.
Pemphigus-associated interstitial lung disease (P-ILD) is a severe complication observed in pemphigus patients that is characterized by pulmonary interstitial inflammation and fibrosis. This study investigated the role of anti-desmoglein (Dsg) 1/3 antibodies in P-ILD pathogenesis and evaluated the therapeutic potential of molecular hydrogen (H). Using a BALB/cJGpt mouse model, we demonstrated that anti-Dsg 1 antibodies, but not anti-Dsg 3 antibodies, induced interstitial inflammation and fibrosis.
View Article and Find Full Text PDFIndian J Dermatol Venereol Leprol
June 2025
Department of Dermatology, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India.
Background Circulating autoantibodies in patients with autoimmune bullous diseases can be detected by indirect immunofluorescence (IIF) microscopy. The sensitivity of this method depends on the substrate used. Normal human skin (NHS) and salt-split skin (SSS) are widely used in conventional serodiagnosis of autoimmune bullous diseases.
View Article and Find Full Text PDFBr J Dermatol
August 2025
Chair of Vegetative Anatomy, Institute of Anatomy, Faculty of Medicine, Ludwig-Maximilians-Universität Munich, Munich, Germany.
Background: Pemphigus vulgaris (PV) is an autoimmune blistering skin disease caused by impaired desmosome adhesion. Altered signalling pathways and direct inhibition of desmoglein (Dsg) binding contribute to loss of cell adhesion, but the sequence of these events is still a matter of debate.
Objectives: To characterize the early sequence of events following autoantibody binding to Dsg3 in the pathogenesis of pemphigus.
J Biol Chem
April 2025
Department of Molecular Biology and Biochemistry, University of California Irvine, Irvine, California, USA.
Patients with pemphigus vulgaris (PV) develop IgG autoantibodies (AuAbs) binding to keratinocyte desmogleins (Dsg), acetylcholine (ACh) receptors, mitochondrial proteins, and some other self-antigens. In this study, we identified linear and discontinuous peptide tetrameric epitope segments (ES) of M3 muscarinic ACh receptor (M3AR) targeted by different anti-M3AR AuAbs. As positive controls, we identified Dsg1 and Dsg3 ES targeted by PV sera.
View Article and Find Full Text PDFJAAD Int
April 2025
Department of Dermatology, University of Lübeck, Lübeck, Germany.
Background: Prospective studies identifying immunological parameters that can predict clinical relapse in pemphigus are scarce.
Objective: To periodically assess immunological parameters in patients with pemphigus vulgaris and foliaceous in remission to understand immunological events preceding clinical relapse.
Methods: A total of 105 patients were included.