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Epidermolysis Bullosa in Spain: Observational Study of a Cohort of Patients Treated in a National Referral Center

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dc.affiliation.dptoUC3M. Departamento de Bioingenieríaes
dc.affiliation.grupoinvUC3M. Grupo de Investigación: Tissue Engineering and Regenerative Medicine (TERMeG)es
dc.contributor.authorMaseda Pedrero, Rocio
dc.contributor.authorQuintana Castañedo, Lucía
dc.contributor.authorPérez Conde, Isabel María
dc.contributor.authorJiménez González, María
dc.contributor.authorEscamez Toledano, Maria Jose
dc.contributor.authorDe Lucas Laguna, Raul
dc.date.accessioned2022-03-23T09:04:58Z
dc.date.available2022-03-23T09:04:58Z
dc.date.issued2021-10-08
dc.description.abstractBackground and objective: Epidermolysis bullosa (EB) is a heterogeneous group of inherited disorders characterized by a high degree of mucocutaneous fragility. This study aimed to describe the clinical and epidemiologic characteristics of patients with EB treated in Hospital Universitario La Paz, a national referral center for inherited EB. Material and methods: Observational, retrospective, single-center study. We included all cases with a clinical and molecular diagnosis of EB managed in the hospital's dermatology department from January 2, 2000, to February 28, 2021. Results: A total of 214 cases were studied. The median (interquartile range) age was 17 (8---32) years; 54.2% were women. One hundred thirty-five (63.1%) patients had dystrophic EB, 67 (31.3%) had EB simplex, 8 (3.7%) had junctional EB, and 3 (1.4%) had Kindler syndrome. One (0.5%) had EB acquisita. Over a third (35.5%) of the patients resided in Madrid. The most common clinical complications were pruritus (63.1%), local infections (56.5%), and pain (54.7%). The most serious ones were cardiomyopathy (in 5.6%) and squamous cell carcinoma (10.3%). Twenty-two patients (10.3%) died.en
dc.format.extent12
dc.identifier.bibliographicCitationMaseda Pedrero, R., Quintana Castanedo, L., Pérez Conde, I., Jiménez González, M., Escámez Toledano, M. J., & de Lucas Laguna, R. (2021). Epidermolysis Bullosa in Spain: Observational Study of a Cohort of Patients Treated in a National Referral Center. In Actas Dermo-Sifiliográficas (English Edition) (Vol. 112, Issue 9, pp. 781–793). Elsevier BV.en
dc.identifier.doihttps://doi.org/10.1016/j.adengl.2021.07.012
dc.identifier.issn1578-2190
dc.identifier.publicationfirstpage781
dc.identifier.publicationissue9
dc.identifier.publicationlastpage793
dc.identifier.publicationtitleActas dermo-sifilográficas (English edition)es
dc.identifier.publicationvolume112
dc.identifier.urihttps://hdl.handle.net/10016/34439
dc.identifier.uxxiAR/0000030385
dc.language.isoengen
dc.publisherELSEVIER BVen
dc.rights© 2021 Published by Elsevier España, S.L.U. on behalf of AEDV.en
dc.rightsAtribución-NoComercial-SinDerivadas 3.0 España*
dc.rights.accessRightsopen accessen
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/3.0/es/*
dc.subject.ecienciaMedicinaes
dc.subject.otherEpidermolysis bullosaen
dc.subject.otherEpidermolysis bullosa simplexen
dc.subject.otherJunctional epidermolysis bullosaen
dc.subject.otherDystrophic epidermolysis bullosaen
dc.subject.otherKindler syndromeen
dc.titleEpidermolysis Bullosa in Spain: Observational Study of a Cohort of Patients Treated in a National Referral Centeren
dc.title.alternativeEpidermólisis bullosa en Espa˜na: Estudio observacional de una cohorte de pacientes atendidos en un centro de referencia nacionales
dc.typeresearch article*
dc.type.hasVersionVoR*
dspace.entity.typePublication
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