Publication: Epidermolysis Bullosa in Spain: Observational Study of a Cohort of Patients Treated in a National Referral Center
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2021-10-08
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ELSEVIER BV
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Abstract
Background 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.
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Epidermolysis bullosa, Epidermolysis bullosa simplex, Junctional epidermolysis bullosa, Dystrophic epidermolysis bullosa, Kindler syndrome
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Maseda 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.