Patients suffering from dystrophic epidermolysis bullosa are prone to developing autoantibodies against skin proteins: A longitudinal confirmational study.
Type VII collagen
autoantibodies
dystrophic epidermolysis bullosa
junctional epidermolysis bullosa
serological follow-up
Journal
Experimental dermatology
ISSN: 1600-0625
Titre abrégé: Exp Dermatol
Pays: Denmark
ID NLM: 9301549
Informations de publication
Date de publication:
Feb 2024
Feb 2024
Historique:
revised:
22
01
2024
received:
23
10
2023
accepted:
04
02
2024
medline:
23
2
2024
pubmed:
23
2
2024
entrez:
23
2
2024
Statut:
ppublish
Résumé
Epidermolysis bullosa (EB) is a heritable skin blistering disease caused by variants in genes coding for proteins that secure cell-cell adhesion and attachment of the epidermis to the dermis. Interestingly, several proteins involved in inherited EB are also associated with autoimmune blistering diseases (AIBD). In this study, we present a long-term follow-up of 15 patients suffering from recessive dystrophic or junctional EB. From these patients, 62 sera were analysed for the presence of autoantibodies associated with AIBD. We show that patients suffering from recessive dystrophic EB (RDEB) are more susceptible to developing autoantibodies against skin proteins than patients suffering from junctional EB (70% vs. 20%, respectively). Interestingly, no correlation with age was observed. Most patients showed reactivity to Type XVII collagen/linear IgA bullous dermatosis autoantigen (n = 5; 33%), followed by BP230 (n = 4; 27%), Type VII collagen (n = 4; 27%) and laminin-332 (n = 1; 7%). The pathogenicity of these autoantibodies remains a subject for future experiments.
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
e15035Subventions
Organisme : Fondo Nacional de Desarrollo Científico y Tecnológico, ANID, Chile
ID : FONDECYT Regular 1220704
Informations de copyright
© 2024 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
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