Epidermólise bolhosa hereditária: perfil clínico-epidemiológico de pacientes atendidos em centro de referência de Aracaju-Sergipe

Abstract

Background: Hereditary epidermolysis bullosa (EB) refers to a group of diseases that present in common characteristics of skin mechanical fragility and the creation of blisters, spontaneously or from minimal traumas. Objective: The present study aimed to evaluate the prevalence of EB, its subtypes and complications, in the state of Sergipe, Brazil. Methods: It`s a case series study, obtained from the analysis of data collected through questionnaire application, medical records and clinical evaluation of patients followed up at a tertiary dermatology service in Sergipe, Brazil. Results: From the total of 12 patients, the majority were male, depended exclusively on care through public health and had its diagnosis in childhood. Half of the sample presented relatives with EB. Among the clinical presentations, dystrophic EB was present in 41.6% of the patients, as well as Simple EB - worth noting that of these, two were of the Dowling Meara subtype. The remaining 16.6% were EB Juncional. All had cicatricial lesions and 66.6% presented mucosal lesions. Study’s limitations: A single evaluation was performed with the patients due to their low locomotion availability. Conclusion: The epidemiology description and main comorbidities associated with EB in Sergipe can serve as a study tool for better adaptation of health measures aimed at this public in the state and at the national level