Epidermolysis bullosa in children: pathophysiology, anaesthesia and analgesia

Abstract

“Few conditions are more devastating to the physical, emotional, and social health of an infant or child than epidermolysis bullosa. The disease is actually a group of congenital abnormalities of the skin, and it encompasses a variety of genetic abnormalities of proteins that mediate adhesion of the skin. These abnormalities result in fragile skin that blisters, causing pain, deformity, social and emotional disability, susceptibility to infection and cancer, and shortened life. Children with epidermolysis bullosa require lifelong support and understanding from physicians (both specialists and g eneralists), from clinical support personnel, and most important, from their families.â€1