{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,26]],"date-time":"2026-06-26T00:46:08Z","timestamp":1782434768626,"version":"3.54.5"},"reference-count":19,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,6,25]],"date-time":"2026-06-25T00:00:00Z","timestamp":1782345600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,6,25]],"date-time":"2026-06-25T00:00:00Z","timestamp":1782345600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Dermatology"],"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background\/Objectives<\/jats:title>\n                    <jats:p>Recessive dystrophic epidermolysis bullosa inversa (RDEB\u2010I) is a rare subtype of dystrophic epidermolysis bullosa (EB) characterized by intertriginous cutaneous involvement and frequent mucosal disease. Although mucosal involvement is recognized in RDEB\u2010I, its cumulative clinical burden remains poorly defined. This study systematically describes mucosal involvement, related complications, and procedural interventions in patients with RDEB\u2010I.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      We performed a retrospective chart review of patients with RDEB\u2010I followed at a multidisciplinary EB center. Records were reviewed to document mucosal involvement across organ systems, associated complications, procedural interventions, and\n                      <jats:italic>COL7A1<\/jats:italic>\n                      variants.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Ten patients were included (mean age at latest follow\u2010up 32.5\u2009years, range 9\u201354; 70% female). Mucosal involvement was universal, with oral, esophageal, and anorectal disease present in all patients (10\/10), and frequent otologic (8\/10), genitourinary (7\/10), and ocular (4\/10) involvement. Patients developed multiple mucosal complications (mean 13.2 per patient; range 8\u201322), requiring numerous procedures (mean 12.4 per patient; range 2\u201330). All involved systems except ocular required procedural intervention, most commonly for esophageal disease, including 74 dilations across 9 patients. Procedural complications occurred in 6 patients, including 10 esophageal mucosal injuries during dilation, as well as corneal abrasions and oropharyngeal injury from anesthetic management. Among patients with available genetic data (9\/10), all harbored at least one variant that is either a glycine or arginine substitution in\n                      <jats:italic>COL7A1<\/jats:italic>\n                      .\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Mucosal disease is a defining feature of RDEB\u2010I, contributing substantially to morbidity and procedural burden across organ systems. Recognition of multisystem mucosal involvement is essential for anticipatory guidance and accurate disease severity assessment.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/pde.70307","type":"journal-article","created":{"date-parts":[[2026,6,25]],"date-time":"2026-06-25T23:49:57Z","timestamp":1782431397000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Multisystem Mucosal Morbidity in Recessive Dystrophic Epidermolysis Bullosa Inversa"],"prefix":"10.1111","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9320-3119","authenticated-orcid":false,"given":"Valerie R.","family":"Stichert","sequence":"first","affiliation":[{"name":"University of Cincinnati College of Medicine  Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nichole","family":"Halliburton","sequence":"additional","affiliation":[{"name":"Division of Dermatology Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Crawford","sequence":"additional","affiliation":[{"name":"Division of Dermatology Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer","family":"Phillips","sequence":"additional","affiliation":[{"name":"Division of Dermatology Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0001-4496-0391","authenticated-orcid":false,"given":"Kalyani S.","family":"Marathe","sequence":"additional","affiliation":[{"name":"University of Cincinnati College of Medicine  Cincinnati Ohio USA"},{"name":"Division of Dermatology Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beth A.","family":"Moeves","sequence":"additional","affiliation":[{"name":"Division of Dermatology Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,6,25]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2010.082230"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jdermsci.2021.09.006"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41439\u2010022\u201000192\u2010y"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365\u20102133.2011.10230.x"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/ced\/llaf009"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/scd.12511"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023\u2010024\u201003434\u20100"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/ced.15029"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajo.2004.03.034"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.ju.0000143200.86683.2c"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2025.5723"},{"issue":"4","key":"e_1_2_11_13_1","first-page":"671","article-title":"Glycine Substitutions in the Triple\u2010Helical Region of Type VII Collagen Result in a Spectrum of Dystrophic Epidermolysis Bullosa Phenotypes and Patterns of Inheritance","volume":"58","author":"Christiano A. 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