{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,30]],"date-time":"2026-04-30T15:36:27Z","timestamp":1777563387043,"version":"3.51.4"},"reference-count":16,"publisher":"SAGE Publications","license":[{"start":{"date-parts":[[2026,1,27]],"date-time":"2026-01-27T00:00:00Z","timestamp":1769472000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,1,27]],"date-time":"2026-01-27T00:00:00Z","timestamp":1769472000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Ear Nose Throat J"],"abstract":"<jats:p>Airway involvement in epidermolysis bullosa (EB) is rare and most commonly associated with junctional EB. Laryngotracheal disease in epidermolysis bullosa simplex (EBS), particularly PLEC (Plectin gene)-related variants, is uncommon but may cause significant morbidity. We report a 7-year-old girl with autosomal recessive PLEC-related EBS who developed progressive supraglottic and glottic stenosis. She presented at 8\u2009months old with severe respiratory distress requiring emergent tracheostomy. Serial endoscopies revealed persistent supraglottic granulation, an anterior glottic web, posterior glottic stenosis, and occasional bullae near the carina, with a patent subglottis and distal trachea. She underwent multiple direct laryngobronchoscopies for cold web release, dilation, mitomycin-C application, Kenacort injection, and excision of suprastomal granulation. Despite clinical stabilization, she remains tracheostomy-dependent with ongoing evaluation for potential decannulation. This case highlights the potential for significant airway disease in PLEC-related EBS, and underscores the importance of early recognition, gentle airway handling, and multidisciplinary management.<\/jats:p>","DOI":"10.1177\/01455613251410928","type":"journal-article","created":{"date-parts":[[2026,1,28]],"date-time":"2026-01-28T00:39:41Z","timestamp":1769560781000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Supraglottic and Glottic Involvement in Epidermolysis Bullosa Simplex: A Pediatric Case Report and Review of Airway Management"],"prefix":"10.1177","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5633-3870","authenticated-orcid":false,"given":"Luluh","family":"Alsughayer","sequence":"first","affiliation":[{"name":"Department of Otolaryngology\u2014Head and Neck Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Abdulrahman","family":"Badghaish","sequence":"additional","affiliation":[{"name":"College of Medicine, King Saud University, Riyadh, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alhanouf","family":"Alhedaithy","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology\u2014Head and Neck Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9872-2711","authenticated-orcid":false,"given":"Zahra","family":"AlMoumen","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology\u2014Head and Neck Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia"},{"name":"Qatif Central Hospital, Eastern Province, Al Qatif, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Abdulmajeed","family":"Zakzouk","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology\u2014Head and Neck Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia"},{"name":"College of Medicine, King Saud University, Riyadh, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2026,1,27]]},"reference":[{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41572-020-0210-0"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2014.01.903"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1097\/MLG.0b013e318093ed8e"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-020-01403-x"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1177\/000348948008900510"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/0165-5876(89)90056-6"},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2006.11.005"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2011.09.029"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.5152\/TJAR.2016.49260"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.14740\/ijcp234w"},{"key":"e_1_3_2_12_2","first-page":"1368","article-title":"Dystrophic epidermolysis bullosa with laryngotracheal involvement: abscess on the vocal cords","volume":"2","author":"Yucel O","year":"2017","unstructured":"Yucel O. Dystrophic epidermolysis bullosa with laryngotracheal involvement: abscess on the vocal cords. Ann Clin Case Rep. 2017;2:1368.","journal-title":"Ann Clin Case Rep"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jdcr.2020.02.033"},{"key":"e_1_3_2_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.xocr.2023.100516"},{"key":"e_1_3_2_15_2","doi-asserted-by":"publisher","DOI":"10.1093\/bjd\/ljac135"},{"issue":"5","key":"e_1_3_2_16_2","first-page":"1","article-title":"Laryngeal lesion of epidermolysis bullosa: topical use of Mitomycin-C","volume":"13","author":"Blandine L","year":"2024","unstructured":"Blandine L, Christine C, Ana G, Anthony L, Sonanda B. Laryngeal lesion of epidermolysis bullosa: topical use of Mitomycin-C. Ann Clin Med Case Rep. 2024;13(5):1-3.","journal-title":"Ann Clin Med Case Rep"},{"issue":"5","key":"e_1_3_2_17_2","first-page":"613","article-title":"Junctional epidermolysis bullosa associated laryngeal stenosis: a case report and review of literature","volume":"36","author":"Balushi F","year":"2024","unstructured":"Balushi F, Al Harrasi Z, Al Farsi M, Al Qurani M. Junctional epidermolysis bullosa associated laryngeal stenosis: a case report and review of literature. Iran J Otorhinolaryngol. 2024;36(5):613-617.","journal-title":"Iran J Otorhinolaryngol"}],"container-title":["Ear, Nose &amp; Throat Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/01455613251410928","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/01455613251410928","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/01455613251410928","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T18:39:20Z","timestamp":1777401560000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/01455613251410928"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,1,27]]},"references-count":16,"alternative-id":["10.1177\/01455613251410928"],"URL":"https:\/\/doi.org\/10.1177\/01455613251410928","relation":{},"ISSN":["0145-5613","1942-7522"],"issn-type":[{"value":"0145-5613","type":"print"},{"value":"1942-7522","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,1,27]]},"article-number":"01455613251410928"}}