TY - JOUR T1 - Epidermolysis Bullosa in Spain: Observational Study of a Cohort of Patients Treated in a National Referral Center JO - Actas Dermo-Sifiliográficas T2 - AU - Maseda Pedrero,R. AU - Quintana Castanedo,L. AU - Pérez Conde,I. AU - Jiménez González,M. AU - Escámez Toledano,M.J. AU - de Lucas Laguna,R. SN - 15782190 M3 - 10.1016/j.adengl.2021.07.012 DO - 10.1016/j.adengl.2021.07.012 UR - https://actasdermo.org/en-epidermolysis-bullosa-in-spain-observational-articulo-S1578219021002122 AB - Background and objectiveEpidermolysis 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 methodsObservational, 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. ResultsA 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. ConclusionsDystrophic EB was the most prevalent clinical form. The most prevalent complications were pruritus, pain, and infections. The most serious ones were cardiomyopathy and squamous cell carcinoma. This study is the first in Spain that explores strategies for improving the health status and quality of life of patients with EB. ER -