{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T18:57:15Z","timestamp":1790621835470,"version":"4.1.0"},"reference-count":29,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2015,11,27]],"date-time":"2015-11-27T00:00:00Z","timestamp":1448582400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Deutsche Derma Gesell"],"published-print":{"date-parts":[[2015,12]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>A genetic blistering skin disease, recessive dystrophic epidermolysis bullosa (RDEB), is marked by severe wound healing defects and finger contractures. The purpose of this investigation was to elucidate the mechanisms of impaired wound healing and pseudosyndactyly occurring in RDEB patients by studying the role of known inflammation and fibrosis markers in RDEB pseudosyndactyly tissue.<\/jats:p><\/jats:sec><jats:sec><jats:title>Patients and methods<\/jats:title><jats:p>We studied the expression of the fibrosis and\/or inflammation markers tenascin\u2010C, \u03b1\u2010smooth muscle actin, transforming growth factor\u2010\u03b21, interleukin\u20101\u03b2, and interleukin\u20106 in scarring and nonscarring tissue from healthy donors and RDEB patients by semiquantitative real time\u2010PCR and, where applicable, by immunoblots. Furthermore, the distribution pattern of \u03b1\u2010smooth muscle actin and tenascin\u2010C were assessed by immunofluorescence microscopy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Based on mRNA and protein analysis, we found upregulation of tenascin\u2010C, interleukin\u20101\u03b2, and interleukin\u20106 \u2013 but not of transforming growth factor\u2010\u03b21 \u2013 in recessive dystrophic epidermolysis bullosa scar samples taken from pseudosyndactyly hands. Unexpectedly, \u03b1\u2010smooth muscle actin was not upregulated.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Our results confirm inflammation and fibrosis in recessive dystrophic epidermolysis bullosa, especially in scars, suggesting major roles for these processes in pseudosyndactyly. Our data therefore suggests the potential use of antiinflammatory and antifibrotic drugs in the prevention of pseudosyndactyly.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ddg.12839","type":"journal-article","created":{"date-parts":[[2015,11,27]],"date-time":"2015-11-27T13:03:59Z","timestamp":1448629439000},"page":"1257-1266","source":"Crossref","is-referenced-by-count":8,"title":["Pseudosyndactyly \u2013 an inflammatory and fibrotic wound healing disorder in recessive dystrophic epidermolysis bullosa"],"prefix":"10.1111","volume":"13","author":[{"given":"Jenny","family":"Breitenbach","sequence":"first","affiliation":[{"name":"Cluster Rheumatology, Balneology and Rehabilitation, Vienna, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christina","family":"Gruber","sequence":"additional","affiliation":[{"name":"Research Program for Molecular Therapy of Genodermatoses EB House Austria Department of Dermatology University Hospital of the Paracelsus Medical University  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