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Die prim\u00e4re und sekund\u00e4re Beteiligung zahlreicher extrakutaner Organsysteme f\u00fchrt zudem zu erheblicher Morbidit\u00e4t und Mortalit\u00e4t. Um diese besser zu bew\u00e4ltigen, sind spezialisierte Behandlungszentren von N\u00f6ten, die eine individuelle, interdisziplin\u00e4r akkordierte Betreuung nach aktuellem medizinischen Standard sowie Zugang zu innovativen Therapiem\u00f6glichkeiten gew\u00e4hrleisten. Mit diesem Ziel wurde 2005 das EB\u2010Haus Austria gegr\u00fcndet, das 2017 zum ersten \u00f6sterreichischen Expertisezentrum f\u00fcr Genodermatosen mit Schwerpunkt auf EB designiert wurde und auch als Mitglied in das Europ\u00e4ische Referenznetzwerk f\u00fcr seltene Hauterkrankungen (ERN Skin) aufgenommen wurde. Die S\u00e4ulen dieser Institution (Ambulanz, Forschungseinheit, Akademie, Klinisches Studienzentrum) betreiben einen regen Austausch untereinander, mit zahlreichen nationalen sowie internationalen klinischen und wissenschaftlichen Partnern, wie auch insbesondere mit Patienten und deren Angeh\u00f6rige \u00fcber die Selbsthilfegruppe DEBRA Austria. Die Entwicklung des EB\u2010Hauses Austria als Referenzzentrum war \u00fcber weite Strecken von Pionierarbeit gekennzeichnet, die wegbereitend f\u00fcr die Optimierung der Betreuung vergleichbarer Erkrankungen sowie allgemeiner Versorgungsstrukturen sein kann.<\/jats:p>","DOI":"10.1111\/ddg.14187_g","type":"journal-article","created":{"date-parts":[[2020,11,30]],"date-time":"2020-11-30T08:43:18Z","timestamp":1606725798000},"page":"1229-1235","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["EB (Epidermolysis bullosa)\u2010Haus Austria: Pionierarbeit f\u00fcr die Betreuung von Patienten mit seltenen Erkrankungen"],"prefix":"10.1111","volume":"18","author":[{"given":"Christine","family":"Prodinger","sequence":"first","affiliation":[{"name":"Universit\u00e4tsklinik f\u00fcr Dermatologie und Allergologie Paracelsus Medizinische Privatuniversit\u00e4t  Salzburg \u00d6sterreich"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin","family":"Laimer","sequence":"additional","affiliation":[{"name":"Universit\u00e4tsklinik f\u00fcr Dermatologie und Allergologie Paracelsus Medizinische Privatuniversit\u00e4t  Salzburg \u00d6sterreich"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Johann W","family":"Bauer","sequence":"additional","affiliation":[{"name":"Universit\u00e4tsklinik f\u00fcr Dermatologie und Allergologie Paracelsus Medizinische Privatuniversit\u00e4t  Salzburg \u00d6sterreich"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Helmut","family":"Hintner","sequence":"additional","affiliation":[{"name":"Universit\u00e4tsklinik f\u00fcr Dermatologie und Allergologie Paracelsus Medizinische Privatuniversit\u00e4t  Salzburg \u00d6sterreich"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2020,11,30]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2014.01.903"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/ddg.40_12774"},{"key":"e_1_2_9_4_1","article-title":"Life with epidermolysis bullosa (EB): etiology, diagnosis, multidisciplinary care and therapy","author":"Fine JD","year":"2008","journal-title":"Wien: Springer"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.18921"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2009.03.052"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2009.03.053"},{"key":"e_1_2_9_8_1","first-page":"1","article-title":"Epidermolysis bullosa: part I \u2013 pathogenesis and clinical features","volume":"28","author":"Murrell D","year":"2010","journal-title":"Dermatologic Clinics"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/1523-1747.ep12541715"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.1982.01650180009008"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/1523-1747.ep12525447"},{"issue":"2","key":"e_1_2_9_12_1","first-page":"127","article-title":"Role of immunofluorescence microscopy in dermatology","volume":"146","author":"Pohla\u2010Gubo G","year":"2011","journal-title":"G Ital Dermatol Venereol"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.det.2009.12.005"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.det.2010.02.008"},{"key":"e_1_2_9_15_1","unstructured":"EUCERD Recommendations on Quality Criteria for Centres of Expertise for RD in MS. 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