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| http://dx.doi.org/10.1016/j.jdcr.2020.04.008 | DOI Listing |
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Associated factors related to production of autoantibodies and dermo-epidermal separation in bullous pemphigoid.
Arch Dermatol Res
January 2025
Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, 9 Dongdan 3rd Alley, Beijing, 100730, China.
Bullous pemphigoid (BP) is a debilitating autoimmune skin blistering disease, characterized by the deposition of specific autoantibodies at the dermal-epidermal junction. This leads to an inflammatory cascade involving the activation of complement proteins, mast cell degranulation, immune cell recruitment, and the release of proteases by granulocytes. While several cytokines and signaling pathways have been implicated in the pathogenesis of BP, the precise mechanism behind autoantibody production remains unclear.
View Article and Find Full Text PDFImmunohistochemistry for Immunoglobulin G4 on Paraffin Sections as a Diagnostic Test for Pemphigus.
Am J Dermatopathol
February 2025
Department of Dermatology and Venereology, Faculty of Medicine, Medical University of Plovdiv, Plovdiv, Bulgaria.
Pemphigus is a group of autoimmune bullous diseases mediated by autoantibodies most often of the immunoglobulin G class, subclasses immunoglobulin G1, and immunoglobulin G4 (IgG4), directed against desmosomal adhesion proteins of keratinocytes. This study aimed to evaluate IgG4 immunoreactivity on paraffin sections using immunohistochemistry in patients with pemphigus as a diagnostic test. Fifty formalin-fixed paraffin-embedded specimens from patients with pemphigus were selected.
View Article and Find Full Text PDFClinical characteristics of bullous pemphigoid patients of different ages and the possible mechanism.
J Dermatol
January 2025
Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, China.
Bullous pemphigoid (BP) is an acquired autoimmune bullous disease that often occurs in elderly patients. Some BP patients with early age of disease onset were observed to have difficulty in receiving applicable disease control. It remains challenging for clinicians to choose the appropriate treatment for these patients.
View Article and Find Full Text PDFOral Cyclosporine Treatment for Four Pediatric Patients With Toxic Epidermal Necrolysis That Showed No Response to High-dose Corticosteroids in Combination With Intravenous Immunoglobulin: A Case Series.
Curr Ther Res Clin Exp
December 2024
Department of Critical Medicine, The Affiliated Hospital of Qingdao University, Qingdao, China.
Background: Immunosuppressive agents like cyclosporine have proven effective in some pediatric cases, although there are limited case reports considering potential risks such as secondary infections.
Objective: This study investigated the safety and efficacy of Cyclosporine A in children who did not respond to high-dose corticosteroids combined with intravenous immunoglobulin (IVIG).
Methods: We reported four pediatric patients diagnosed with toxic epidermal necrolysis (TEN) received treatment at our institution.
Clinical Features of Chinese Patients With Bullous Pemphigoid Induced by Immune Checkpoint Inhibitors.
Zhongguo Yi Xue Ke Xue Yuan Xue Bao
December 2024
Department of Dermatology,State Key Laboratory of Complex Severe and Rare Diseases,PUMC Hospital,CAMS and PUMC, National Clinical Research Center for Dermatologic and Immunologic Diseases,Beijing 100730,China.
Objective To explore the clinical features and treatments of Chinese patients with bullous pemphigoid (BP) induced by immune checkpoint inhibitors (ICI). Methods A retrospective analysis was conducted on 18 Chinese patients with ICI-induced BP treated in the Peking Union Medical College Hospital and 14 Chinese patients with this disease reported in the literature.Furthermore,the research data of non-Chinese patients were used for comparison to outline the clinical features and treatment responses of the Chinese patients.
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