{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T04:47:45Z","timestamp":1784868465182,"version":"3.55.0"},"reference-count":5,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2021,9,13]],"date-time":"2021-09-13T00:00:00Z","timestamp":1631491200000},"content-version":"vor","delay-in-days":12,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100002077","name":"Epidermolysis Bullosa Medical Research Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100002077","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Dermatology"],"published-print":{"date-parts":[[2021,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Dystrophic epidermolysis bullosa is a debilitating skin condition, without curative treatment. Previous research has focused on the recessive variant, which is known to cause severe disease. Limited work focusing on the clinical manifestations and outcomes of dominant dystrophic epidermolysis bullosa is found (DDEB).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Analysis of an online survey of 42 DDEB patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Self\u2010reported severity of disease did not correlate with size of the wound or number of dressing changes, but did correlate with severity of pain reported in the last 12\u00a0months (3.4 mild vs 6.8 severe disease, <jats:italic>P<\/jats:italic>\u00a0=\u00a00.0002). Patients with severe DDEB also reported more severe internal disease symptoms, such as difficulty swallowing (62.5%, <jats:italic>P<\/jats:italic>\u00a0=\u00a00.01) and greater analgesic use during dressing changes (4.4% mild vs 81.3% severe, <jats:italic>P<\/jats:italic>\u00a0=\u00a0&lt;0.001).<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>Patient perception of disease severity in DDEB appears to be most impacted by pain, presence of chronic open wounds, difficulty swallowing, difficulty walking, and anal strictures. As research on DDEB increases, future studies focused on these symptoms might be the most impactful for DDEB patients. However, distinguishing DDEB from other subtypes remains a challenge.<\/jats:p><\/jats:sec>","DOI":"10.1111\/pde.14802","type":"journal-article","created":{"date-parts":[[2021,9,13]],"date-time":"2021-09-13T10:53:25Z","timestamp":1631530405000},"page":"1198-1201","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Patient\u2010reported outcomes and quality of life in dominant dystrophic epidermolysis bullosa: A global cross\u2010sectional survey"],"prefix":"10.1111","volume":"38","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2918-3931","authenticated-orcid":false,"given":"Shivali","family":"Fulchand","sequence":"first","affiliation":[{"name":"Department of Dermatology Stanford University School of Medicine Stanford CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole","family":"Harris","sequence":"additional","affiliation":[{"name":"Department of Dermatology Stanford University School of Medicine Stanford CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shufeng","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Dermatology Stanford University School of Medicine Stanford CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melissa","family":"Barriga","sequence":"additional","affiliation":[{"name":"Department of Dermatology Stanford University School of Medicine Stanford CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2813-3089","authenticated-orcid":false,"given":"Emily","family":"Gorell","sequence":"additional","affiliation":[{"name":"Department of Dermatology University of Cincinnati College of Medicine Cincinnati OH USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"De Souza","sequence":"additional","affiliation":[{"name":"deSouzaTech, LLC Berkeley CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2971-0199","authenticated-orcid":false,"given":"Dedee","family":"Murrell","sequence":"additional","affiliation":[{"name":"The George Institute of Global Health University of New South Wales Sydney NSW Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. Peter","family":"Marinkovich","sequence":"additional","affiliation":[{"name":"Department of Dermatology Stanford University School of Medicine Stanford CA USA"},{"name":"Veterans Affairs Medical Center Palo Alto CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vamsi","family":"Krishna Yenamandra","sequence":"additional","affiliation":[{"name":"Skin &amp; Epithelial Cell Biology Unit CSIR\u2010Institute of Genomics &amp; Integrative Biology New Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean Y.","family":"Tang","sequence":"additional","affiliation":[{"name":"Department of Dermatology Stanford University School of Medicine Stanford CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,9,13]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.18921"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jid.2020.08.018"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2020.03.028"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/s0945\u2010053x(98)00007\u20109"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2018.4673"}],"updated-by":[{"DOI":"10.1111\/pde.15081","type":"erratum","label":"Erratum","source":"publisher","updated":{"date-parts":[[2022,11,28]],"date-time":"2022-11-28T00:00:00Z","timestamp":1669593600000}}],"container-title":["Pediatric Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pde.14802","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/pde.14802","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pde.14802","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,5]],"date-time":"2023-10-05T20:06:50Z","timestamp":1696536410000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/pde.14802"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,9]]},"references-count":5,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2021,9]]}},"alternative-id":["10.1111\/pde.14802"],"URL":"https:\/\/doi.org\/10.1111\/pde.14802","archive":["Portico"],"relation":{"erratum":[{"id-type":"doi","id":"10.1111\/pde.15081","asserted-by":"object"}]},"ISSN":["0736-8046","1525-1470"],"issn-type":[{"value":"0736-8046","type":"print"},{"value":"1525-1470","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,9]]},"assertion":[{"value":"2021-09-13","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}