EPIDERMMOLYSIS BULLOSA: A COMPREHENSIVE APPROACH ON ITS ETIOPATHOGENESIS, CLINICAL IMPLICATIONS AND TREATMENT

• DOI: https://doi.org/10.22533/at.ed.1594512405063

• Palavras-chave: Epidermolysis Bullosa, mechano-bullous dermatosis, genodermatosis

• Keywords: Epidermolysis Bullosa, mechano-bullous dermatosis, genodermatosis

• Abstract:

  Introduction: Epidermolysis Bullosa (EB) is a congenital, rare, chronic, systemic, serious, non-contagious and autoimmune condition, considered a mechano-bullous dermatosis. Characteristically, affected individuals develop vesicles or blisters on the skin and mucous membranes, spontaneously or after minimal trauma. Therefore, EB appears to be a relevant topic for scientific investigations. Objective: Understand the pathophysiological aspects, causes, symptoms, diagnosis and treatment of epidermolysis bullosa, as well as its clinical implications. Methodology: The present study is a bibliographic review article related to Epidermolysis Bullosa, carried out between June and August 2024, 9 articles were selected, in which the review of articles was carried out in the electronic library database Scientific Eletronic Library Online ( SCIELO), Journal of the USP School of Nursing, Latin American and Caribbean Literature in Health Sciences (LILACS), ``Revista de Enfermagem do Centro-Oeste Mineiro``, Estima–Brazilian Journal of Enterostomal Therapy, ``Revista Portuguesa de estomatologia``, Dental medicine and Maxillofacial Surgery, Electronic Annals, IX EPCC–International Meeting of Scientific Production UniCesumar and PubMed Central (PMC). The following selection criteria were defined: full texts, books, analysis and systematic reviews, between 2008 and 2021, in Portuguese and English. Results: EB is a disease associated with skin fragility, which is why its sufferers generally present vesicles or blisters on the skin and mucous membranes, spontaneously or after minimal trauma. It appears that the main manifestations are oral, ranging from the formation of blisters on the mucosa to erosions and denudation of the tongue and mucous membranes. 

  For this reason, it is essential that dental follow-up is carried out early in patients with EB, so that it occurs in a preventive and resolutive manner. However, its treatment is not restricted to dentists alone, it occurs in a multidisciplinary manner, involving psychologists, nutritionists, physiotherapists, hematologists, dermatologists and nurses. In it, palliative measures are used to minimize risks such as infection, formation of exudate, foul odor, and ineffectiveness in preventing future wounds on delicate skin. It is also known that the better the treatment, the better the quality of life of those with EB. Regarding the prognosis, it varies according to the type of EB, the time for diagnosis and the correct treatment. In general, individuals with EB have a normal life expectancy. Conclusions: It is concluded that EB has multiple and major impacts on the lives of its sufferers and therefore requires early diagnosis and adequate and effective treatment in order to reduce its complications and improve the quality of life of patients.