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Paw Skin as a Translational Model for Investigating Fibrotic and Inflammatory Wound Healing Defects in Recessive Dystrophic Epidermolysis Bullosa

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dc.contributor.authorDe Gregorio, Cristian
dc.contributor.authorRamos, Giselle
dc.contributor.authorMorales, Bernardo
dc.contributor.authorEzquer, Fernando
dc.contributor.authorMarcelo Ezquer
dc.contributor.authorEzquer, Marcelo
dc.date.accessioned2026-08-13T21:12:12Z
dc.date.available2026-08-13T21:12:12Z
dc.date.issued2025
dc.description.abstractRecessive dystrophic epidermolysis bullosa (RDEB) is a severe genetic disease caused by COL7A1 mutations. It leads to skin fragility, chronic inflammation, and impaired wound healing. The condition often results in fibrotic scarring, pseudosyndactyly, and cutaneous squamous cell carcinoma (SCC). However, current animal models fail to fully replicate chronic RDEB wounds. In this study, we used Collagen VII-hypomorphic mice (Col7a1flNeo/flNeo) and created full-thickness wounds on their paw skin, an area prone to fibrosis due to mechanical stress. We analyzed the healing process using histology, immunofluorescence, and electron microscopy. The RDEB mice showed delayed wound closure, increased inflammation, and poor granulation tissue formation. At 30 days post-injury, we observed persistent fibrosis, with elevated levels of Collagen I, α-SMA+ myofibroblasts, and tenascin-C. These mice also had fewer intraepidermal nerve fibers, which may help explain the neuropathic pain associated with RDEB. Our model reproduces the main features of chronic RDEB wounds. It offers a useful tool for evaluating therapies aimed at reducing inflammation, fibrosis, and tumor risk in these patients.
dc.description.versionVersión Publicada
dc.identifier.citationDe Gregorio C, Ramos-Gonzalez G, Morales-Catalán B, Ezquer F, Ezquer M. Paw Skin as a Translational Model for Investigating Fibrotic and Inflammatory Wound Healing Defects in Recessive Dystrophic Epidermolysis Bullosa. Int J Mol Sci. 2025 Apr 30;26(9):4281. doi: 10.3390/ijms26094281
dc.identifier.doihttps://doi.org/10.3390/ijms26094281
dc.identifier.urihttps://hdl.handle.net/11447/11010
dc.language.isoen
dc.subjectRDEB
dc.subjectinflammation
dc.subjectpaw skin model
dc.subjectsensory nerve alteration
dc.subjectskin fibrosis
dc.subjectwound healing
dc.titlePaw Skin as a Translational Model for Investigating Fibrotic and Inflammatory Wound Healing Defects in Recessive Dystrophic Epidermolysis Bullosa
dc.typeArticle
dcterms.accessRightsAcceso Abierto
dcterms.sourceInternational journal of molecular sciences
dspace.entity.typePublication
relation.isAuthorOfPublicationc74766c7-dad8-4d11-b35d-01da0664f3da
relation.isAuthorOfPublication9cd4a8e6-6ba1-4344-80a2-45c260864d4c
relation.isAuthorOfPublication.latestForDiscoveryc74766c7-dad8-4d11-b35d-01da0664f3da

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