Patients suffering from dystrophic epidermolysis bullosa are prone to developing autoantibodies against skin proteins: A longitudinal confirmational study

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dc.contributor.authorBremer, J.
dc.contributor.authorPas, H. H.
dc.contributor.authorDiercks, G. F. H.
dc.contributor.authorMeijer, H. J.
dc.contributor.authorvan Der Molen, S. M.
dc.contributor.authorNijenhuis, A. M.
dc.contributor.authorvan Nijen-Vos, L. L.
dc.contributor.authorMorande, P.
dc.contributor.authorYubero, M. J.
dc.contributor.authorPalisson, F.
dc.contributor.authorFuentes, I.
dc.contributor.authorPasmooij, A. M. G.
dc.date.accessioned2025-01-20T17:06:04Z
dc.date.available2025-01-20T17:06:04Z
dc.date.issued2024
dc.description.abstractEpidermolysis 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.
dc.fuente.origenWOS
dc.identifier.doi10.1111/exd.15035
dc.identifier.eissn1600-0625
dc.identifier.issn0906-6705
dc.identifier.urihttps://doi.org/10.1111/exd.15035
dc.identifier.urihttps://repositorio.uc.cl/handle/11534/90749
dc.identifier.wosidWOS:001169614200001
dc.issue.numero2
dc.language.isoen
dc.revistaExperimental dermatology
dc.rightsacceso restringido
dc.subjectautoantibodies
dc.subjectdystrophic epidermolysis bullosa
dc.subjectjunctional epidermolysis bullosa
dc.subjectserological follow-up
dc.subjectType VII collagen
dc.subject.ods03 Good Health and Well-being
dc.subject.odspa03 Salud y bienestar
dc.titlePatients suffering from dystrophic epidermolysis bullosa are prone to developing autoantibodies against skin proteins: A longitudinal confirmational study
dc.typeartículo
dc.volumen33
sipa.indexWOS
sipa.trazabilidadWOS;2025-01-12
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