{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T17:21:12Z","timestamp":1791652872450,"version":"4.3.4"},"reference-count":21,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2016,10,3]],"date-time":"2016-10-03T00:00:00Z","timestamp":1475452800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100005107","name":"Australasian Blistering Diseases Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005107","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Acad Dermatol Venereol"],"published-print":{"date-parts":[[2017,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>The lack of validated outcome measures for epidermolysis bullosa (<jats:styled-content style=\"fixed-case\">EB<\/jats:styled-content>) presents major barriers to evaluating disease severity and comparing the efficacy of therapies. The Epidermolysis Bullosa Disease Activity and Scarring Index (<jats:styled-content style=\"fixed-case\">EBDASI<\/jats:styled-content>) was recently introduced as a valid and reliable instrument for <jats:styled-content style=\"fixed-case\">EB<\/jats:styled-content>; however, its interpretation for use in clinical practice and clinical trials is yet to be defined.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>To assess the interpretability of the <jats:styled-content style=\"fixed-case\">EBDASI<\/jats:styled-content> in classifying patients according to disease severity and clinical response.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A total of 53 outpatients with <jats:styled-content style=\"fixed-case\">EB<\/jats:styled-content> at two interstate institutions were prospectively evaluated. At each visit, the principal dermatologist completed the <jats:styled-content style=\"fixed-case\">EBDASI<\/jats:styled-content> and global assessments of disease severity and change. Classifications for mild, moderate and severe disease using the <jats:styled-content style=\"fixed-case\">EBDASI<\/jats:styled-content> were determined using receiver operating characteristic curves. Minimal clinically important differences for the <jats:styled-content style=\"fixed-case\">EBDASI<\/jats:styled-content> activity subscale were calculated and compared with the standard error of measurement.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Total <jats:styled-content style=\"fixed-case\">EBDASI<\/jats:styled-content> score ranges of 0\u201342, 43\u2013106 and 107\u2013506 corresponded to mild, moderate and severe disease respectively. Reduction in <jats:styled-content style=\"fixed-case\">EBDASI<\/jats:styled-content> activity scores of greater than 9 indicated clinically significant improvement. An increase of 3 in the activity score indicated deterioration.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The <jats:styled-content style=\"fixed-case\">EBDASI<\/jats:styled-content> is a responsive tool and may be useful in characterizing disease severity and response. The cut\u2010offs proposed in this study provide the first practical guide for interpreting the <jats:styled-content style=\"fixed-case\">EBDASI<\/jats:styled-content>, further supporting its use for longitudinal patient assessment and in clinical trials.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jdv.13953","type":"journal-article","created":{"date-parts":[[2016,8,31]],"date-time":"2016-08-31T18:31:39Z","timestamp":1472668299000},"page":"692-698","source":"Crossref","is-referenced-by-count":43,"title":["The Epidermolysis Bullosa Disease Activity and Scarring Index (<scp>EBDASI<\/scp>): grading disease severity and assessing responsiveness to clinical change in epidermolysis bullosa"],"prefix":"10.1111","volume":"31","author":[{"given":"S.V.","family":"Jain","sequence":"first","affiliation":[{"name":"Department of Dermatology St George Hospital Sydney NSW Australia"},{"name":"Faculty of Medicine University of New South Wales Sydney NSW Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.G.","family":"Harris","sequence":"additional","affiliation":[{"name":"Department of Dermatology St George Hospital Sydney NSW Australia"},{"name":"Faculty of Medicine University of New South Wales Sydney NSW Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.C.","family":"Su","sequence":"additional","affiliation":[{"name":"Department of Dermatology The Royal Children's Hospital Melbourne Vic. 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Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.J.","family":"Warren","sequence":"additional","affiliation":[{"name":"Department of Dermatology Women's and Children's Hospital Adelaide SA Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H.","family":"McManus","sequence":"additional","affiliation":[{"name":"Kirby Institute University of New South Wales Sydney NSW Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.F.","family":"Murrell","sequence":"additional","affiliation":[{"name":"Department of Dermatology St George Hospital Sydney NSW Australia"},{"name":"Faculty of Medicine University of New South Wales Sydney NSW Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,10,3]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00403-002-0379-y"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.2009.09041.x"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/pde.12317"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2013.09.041"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2009.72"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.0022-202X.2005.23889.x"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1038\/jid.2011.440"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.4065\/77.4.371"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/0197-2456(89)90005-6"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1001\/archdermatol.2010.435"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.2009.09347.x"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.2340\/00015555-1758"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0895-4356(99)00206-1"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0895-4356(99)00071-2"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2230.2004.01428.x"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.2340\/00015555-0376"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jclinepi.2007.03.012"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jclinepi.2008.07.009"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0895-4356(01)00407-3"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.12599"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2013.08.014"}],"container-title":["Journal of the European Academy of Dermatology and Venereology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fjdv.13953","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fjdv.13953","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/jdv.13953","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,23]],"date-time":"2023-09-23T13:39:42Z","timestamp":1695476382000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/jdv.13953"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,10,3]]},"references-count":21,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2017,4]]}},"alternative-id":["10.1111\/jdv.13953"],"URL":"https:\/\/doi.org\/10.1111\/jdv.13953","archive":["Portico"],"relation":{},"ISSN":["0926-9959","1468-3083"],"issn-type":[{"value":"0926-9959","type":"print"},{"value":"1468-3083","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,10,3]]}}}