Prevenção e tratamento tópico de lesão decorrente de epidermólise bolhosa baseados em evidência

Abstract

This work is primarily intended to bring detailed information about Epidermolysis Bullosa to the attention of nursing professionals. EB (Epidermolysis Bullosa) is a rare, and very serious, congenital disease, transmitted from parent to offspring, caused by a deficiency in the activity of the collagenase enzyme and characterized by the formation of blisters in the dermoepidermal junction. This is an illness which has only been studied intermittently and without any great hope of finding a cure. It complicates even the smallest skin trauma, affects various functions of the body and requires special care which, most of the time, is either unknown to, or ignored by, the nursing team. The objectives of this work, therefore, are to provide recommendations for the prevention of traumas in carriers of Epidermolysis Bullosa and to identify the topical treatment of EB skin wounds based on scientific evidence. This offers the possibility of finding a way to avoid aggravating the wounds using specific therapeutical measures whilst also demonstrating the importance of interaction between the nurses and the other health professionals in order to determine the most appropriate treatment - thereby minimising the intense suffering of the patients concerned. On the basis of a review of the specialist literature, as well as day to day familiarity with children carrying this illness, a basic set of procedures and recommendations are here presented with a view to improve the quality of life of these patients and to provide useful guidance for members of their families.