{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T01:38:07Z","timestamp":1790127487690,"version":"4.0.1"},"reference-count":45,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2025,1,24]],"date-time":"2025-01-24T00:00:00Z","timestamp":1737676800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100019725","name":"DEBRA Austria","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100019725","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Dermatology"],"published-print":{"date-parts":[[2025,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Recessive dystrophic epidermolysis bullosa (RDEB) is caused by mutations in <jats:italic>COL7A1<\/jats:italic>, leading to loss or dysfunction of type\u2010VII collagen (C7), a protein essential for skin stability. Clinically, patients suffer from severe skin blistering, chronic or recurrent wounds, and scarring, which predispose to early onset of aggressive squamous cell carcinoma. Previous studies showed that RDEB\u2010keratinocytes (RDEB\u2010KC) express high levels of matrix\u2010metalloproteinase 9 (MMP\u20109), a molecule known to play a crucial role in wound chronification if dysregulated. We investigated the potential of diacerein, a small molecule that interferes with the MMP\u20109 regulatory pathway, to improve wound healing in a 5\u2010year old RDEB patient presenting with chronic, generalized skin involvement unresponsive to previous treatment approaches. Upon 4\u2009weeks of topical therapy applied to the patient's back, parents reported a nearly complete wound closure and a significant increase in quality of life. We also provide evidence that diacerein treatment of patient keratinocytes results in a downregulation of MMP\u20109 expression, accompanied by a reduction in their ability to degrade a fibrinogen matrix. These data characterize diacerein as a potential candidate for improving wound healing in RDEB through its impact on inflammatory as well as epithelial cells.<\/jats:p>","DOI":"10.1111\/1346-8138.17621","type":"journal-article","created":{"date-parts":[[2025,1,24]],"date-time":"2025-01-24T10:47:16Z","timestamp":1737715636000},"page":"423-431","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Repurposing diacerein for the treatment of chronic wounds in recessive\u2010dystrophic epidermolysis bullosa patients by modulating matrix metalloproteinase\u20109 expression"],"prefix":"10.1111","volume":"52","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8286-9620","authenticated-orcid":false,"given":"Sonja","family":"Dorfer","sequence":"first","affiliation":[{"name":"Department of Dermatology and Allergology, EB House Austria, Research Program for Molecular Therapy of Genodermatoses University Hospital of the Paracelsus Medical University Salzburg  Salzburg Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Ablinger","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Allergology, EB House Austria, Research Program for Molecular Therapy of Genodermatoses University Hospital of the Paracelsus Medical University Salzburg  Salzburg Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monika","family":"Wimmer","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Allergology, EB House Austria, Research Program for Molecular Therapy of Genodermatoses University Hospital of the Paracelsus Medical University Salzburg  Salzburg Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia I.","family":"Hummel","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Allergology, EB House Austria, Research Program for Molecular Therapy of Genodermatoses University Hospital of the Paracelsus Medical University Salzburg  Salzburg Austria"},{"name":"Department of Biosciences and Medical Biology University of Salzburg  Salzburg Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Selma","family":"Ibrahimpa\u0161i\u0107","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Allergology, EB House Austria, Research Program for Molecular Therapy of Genodermatoses University Hospital of the Paracelsus Medical University Salzburg  Salzburg Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anja","family":"Diem","sequence":"additional","affiliation":[{"name":"Department of Dermatology and Allergology, EB House Austria, Research Program for Molecular Therapy of Genodermatoses University Hospital of the Paracelsus Medical University Salzburg  Salzburg 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