Background: Bullous pemphigoid (BP) is an autoimmune blistering dermatosis associated with a number of neurological conditions, including idiopathic Parkinson's disease (IPD). Only 1 case of BP in a patient with multiple system atrophy (MSA) has been reported.
Cases: We report 3 cases of men with probable MSA who developed bullous pemphigoid at a latency of 4-6 years from MSA symptom onset.
Conclusions: Skin α-synuclein deposition in neurodegenerative conditions such as IPD and MSA may be a potential substrate for the exposure of BP-related antigens. Alternatively, central neurodegeneration may expose antigens as a substrate for cross-reactivity and BP pathogenesis. Our report suggests an association between BP and MSA, in addition to the previously documented association with IPD.
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http://dx.doi.org/10.1002/mdc3.13160 | DOI Listing |
J Eur Acad Dermatol Venereol
February 2025
2nd Dermatology Department, Center of Expertise on AIBD, Papageorgiou General Hospital, Aristotle University School of Medicine, Thessaloniki, Greece.
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 PDFAm 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 PDFChin Med J (Engl)
January 2025
Hospital for Skin Diseases, Shandong First Medical University, Jinan, Shandong 250061, China.
Oxf Med Case Reports
January 2025
Department of Internal Medicine, Faculty of Medicine, Hasanuddin University, Jalan Perintis Kemerdekaan KM. 11, Makassar, South Sulawesi 90245, Indonesia.
Historically, adolescents and young adults diagnosed with acute lymphoblastic leukemia (ALL) have faced lower survival rates compared to children with the same illness. Bullous pemphigoid (BP), a rare autoimmune skin disorder, poses unique challenges when occurring alongside hematologic malignancies. A 23-year-old male with ALL-L1 diagnosis who developed bullous pemphigoid in this report.
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