{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T07:04:59Z","timestamp":1789715099552,"version":"4.0.1"},"reference-count":17,"publisher":"Ovid Technologies (Wolters Kluwer Health)","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"abstract":"<jats:sec>\n                    <jats:title>Objectives:<\/jats:title>\n                    <jats:p>To describe the clinical characteristics, reasons for consultation, management, and outcomes of patients with epidermolysis bullosa (EB) presenting to a pediatric emergency department (PED).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>We conducted a retrospective, observational study in a tertiary-care PED in Spain, designated as a national reference center for EB. All visits by patients with a confirmed diagnosis of EB between 2011 and 2025 were included. Demographic, clinical, and management data were collected.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      Twenty-nine patients accounted for 169 PED visits (median age: 8.1\u00a0y; 62% female). Recessive dystrophic EB was the most frequent subtype (72.4%). The median number of visits per patient was 2 (IQR: 1 to 9). Most visits were triaged as level 3 (68%). The leading reasons for consultation were infections (42.6%), gastrointestinal complications (22.5%), particularly esophageal involvement, and gastrostomy-related problems (14.2%). Additional tests were required in 57.4% of visits. Wound\/skin cultures were positive in 83.3% of cases in which they were obtained, most commonly isolating Gram-negative organisms and\n                      <jats:italic toggle=\"yes\">Staphylococcus<\/jats:italic>\n                      species. Anemia was present in 83.3% of cases in which hemoglobin was measured. In patients with sepsis, both C-reactive protein and procalcitonin levels were significantly higher compared with non-sepsis cases. Venous access was required in 33.1% of visits and was difficult in 23.2%. Systemic antibiotics were administered in 35.5% of episodes, frequently intravenously. Hospital admission was required in 31% of visits, including 4.7% to the pediatric intensive care unit. Two deaths were recorded.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>Pediatric patients with EB frequently present to the PED with complex and potentially severe conditions, particularly infections and gastrointestinal complications. High rates of anemia, need for intravenous therapy, and difficulties in vascular access highlight the challenges of acute management. Improved clinician training and standardized care pathways may enhance the safety and quality of emergency care for this vulnerable population.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1097\/pec.0000000000003703","type":"journal-article","created":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T07:00:09Z","timestamp":1789714809000},"update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":0,"title":["Pediatric Patients With Epidermolysis Bullosa in the Emergency Department"],"prefix":"10.1097","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4866-6656","authenticated-orcid":false,"given":"Paula","family":"Garc\u00eda-S\u00e1nchez","sequence":"first","affiliation":[{"name":"Pediatric Emergency"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beatriz","family":"Jim\u00e9nez de la Jara","sequence":"additional","affiliation":[{"name":"Pediatric Emergency"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roc\u00edo","family":"Maseda Pedrero","sequence":"additional","affiliation":[{"name":"Dermatology. La Paz University Hospital, Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ra\u00fal","family":"de Lucas Laguna","sequence":"additional","affiliation":[{"name":"Dermatology. La Paz University Hospital, Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosario","family":"L\u00f3pez L\u00f3pez","sequence":"additional","affiliation":[{"name":"Pediatric Emergency"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mar\u00eda","family":"de Ceano-Vivas la Calle","sequence":"additional","affiliation":[{"name":"Pediatric Emergency"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2026,9,18]]},"reference":[{"key":"R1-20260918","first-page":"00176","article-title":"Epidermolysis bullosa in Spain: observational study of a cohort of patients treated in a National Referral Center","volume":"S0001-7310","author":"Maseda Pedrero","year":"2021","journal-title":"Actas Dermosifiliogr"},{"key":"R2-20260918","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1186\/s13023-020-01403-x","article-title":"Emergency management in epidermolysis bullosa: consensus clinical recommendations from the European reference network for rare skin diseases","volume":"15","author":"Mellerio","year":"2020","journal-title":"Orphanet J Rare Dis"},{"key":"R3-20260918","doi-asserted-by":"crossref","first-page":"182","DOI":"10.2174\/1573396317666210525161252","article-title":"Epidermolysis bullosa: pediatric perspectives","volume":"18","author":"Hon","year":"2022","journal-title":"Curr Pediatr Rev"},{"key":"R4-20260918","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1146\/annurev-genom-090413-025540","article-title":"The genetics of skin fragility","volume":"15","author":"Has","year":"2014","journal-title":"Annu Rev Genomics Hum Genet"},{"key":"R5-20260918","doi-asserted-by":"crossref","first-page":"614","DOI":"10.1111\/bjd.18921","article-title":"Consensus reclassification of inherited epidermolysis bullosa and other disorders with skin fragility","volume":"183","author":"Has","year":"2020","journal-title":"Br J Dermatol"},{"key":"R6-20260918","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1016\/j.det.2009.10.020","article-title":"Dystrophic epidermolysis bullosa: pathogenesis and clinical features","volume":"28","author":"Bruckner-Tuderman","year":"2010","journal-title":"Dermatol Clin"},{"key":"R7-20260918","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1097\/PEC.0000000000001500","article-title":"Caring for pediatric patients with epidermolysis bullosa in the emergency department","volume":"35","author":"Jenkinson","year":"2019","journal-title":"Pediatr Emerg Care"},{"key":"R10-20260918","first-page":"214","article-title":"Anemias. Clasificaci\u00f3n y diagn\u00f3stico","volume":"XXV","author":"Rosich del Cacho","year":"2021","journal-title":"Pediatr Integral"},{"key":"R11-20260918","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1186\/s13023-021-02144-1","article-title":"Epidermolysis bullosa in children: the central role of the pediatrician","volume":"17","author":"Marchili","year":"2022","journal-title":"Orphanet J Rare Dis"},{"key":"R12-20260918","doi-asserted-by":"crossref","first-page":"106452","DOI":"10.1016\/j.nedt.2024.106452","article-title":"Optimizing triage education for emergency room nurses: a scoping review","volume":"144","author":"Shin","year":"2025","journal-title":"Nurse Educ Today"},{"key":"R13-20260918","doi-asserted-by":"crossref","first-page":"101210","DOI":"10.1016\/j.jhqr.2026.101210","article-title":"Impacto de la implantaci\u00f3n de una herramienta de identificaci\u00f3n precoz en la atenci\u00f3n en urgencias de pacientes oncol\u00f3gicos pedi\u00e1tricos con neutropenia febril","volume":"41","author":"Garc\u00eda-S\u00e1nchez","year":"2026","journal-title":"J Healthc Qual Res"},{"key":"R14-20260918","doi-asserted-by":"crossref","first-page":"101811","DOI":"10.1016\/j.semerg.2022.06.001","article-title":"Tarjetas de alerta para enfermedades rearas","volume":"49","author":"Rosado-Bartolom\u00e9","year":"2023","journal-title":"Semergen"},{"key":"R15-20260918","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1111\/j.1525-1470.2009.01070.x","article-title":"Common wound colonizers in patients with epidermolysis bullosa","volume":"27","author":"Brandling-Bennett","year":"2010","journal-title":"Pediatr Dermatol"},{"key":"R16-20260918","doi-asserted-by":"crossref","first-page":"544","DOI":"10.1016\/j.ad.2017.01.015","article-title":"Cardiomyopathy in patients with hereditary bullous epidermolysis","volume":"108","author":"Batalla","year":"2017","journal-title":"Actas Dermosifiliogr"},{"key":"R17-20260918","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1186\/s13023-025-04034-2","article-title":"Tissue and systemic inflammation in dystrophic epidermolysis bullosa: a systematic review and meta-analysis","volume":"20","author":"Karakioulaki","year":"2025","journal-title":"Orphanet J Rare Dis"},{"key":"R18-20260918","doi-asserted-by":"crossref","first-page":"e15146","DOI":"10.1111\/exd.15146","article-title":"Unveiling the value of C-reactive protein as a severity biomarker and the IL4\/IL13 pathway as a therapeutic target in recessive dystrophic epidermolysis bullosa: a multiparametric cross-sectional study","volume":"33","author":"Quintana-Castanedo","year":"2024","journal-title":"Exp Dermatol"},{"key":"R19-20260918","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1177\/08850666231199050","article-title":"Difficult intravascular access in pediatric emergency department: the ultrasound-assisted strategy (DIAPEDUS Study)","volume":"39","author":"D\u2019Alessandro","year":"2024","journal-title":"J Intensive Care Med"}],"container-title":["Pediatric Emergency Care"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.1097\/PEC.0000000000003703","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T07:00:12Z","timestamp":1789714812000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.1097\/PEC.0000000000003703"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,18]]},"references-count":17,"URL":"https:\/\/doi.org\/10.1097\/pec.0000000000003703","relation":{},"ISSN":["0749-5161","1535-1815"],"issn-type":[{"value":"0749-5161","type":"print"},{"value":"1535-1815","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9,18]]},"assertion":[{"value":"2026-04-28","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-08-28","name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}}]}}