oa Southern African Journal of Anaesthesia and Analgesia - Epidermolysis bullosa in children : pathophysiology, anaesthesia and analgesia : paediatric anaesthesia
Epidermolysis bullosa (EB) is a rare, genetically determined mucocutaneous, mechanobullous group of disorders. Three types have been described, but all show the same tendency for the skin or mucous membranes to separate from the underlying tissue, with minimal mechanical trauma, resulting in blistering. The disease runs a very variable course from minor disability, to those patients who have a longer life-span with pain and suffering, and to death in infancy. The cycle of trauma, secondary infection, healing, scarring, and deformity forms the pattern of their lives. Over time, many physical and emotional complications arise as these patients, because of their disabilities, face social challenges, their exclusion from normal activities and, because of frequent hospital visits, time away from school.
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