Epidermolysis bullosa: pathophysiology and nursing care

Abstract

Epidermolysis bullosa (EB) is defined as a group of skin disorders that presents as blistering of the skin in varying degrees of severity. Most cases are inherited, but rare cases may be acquired. There are three main types: simplex, junctional, and dystrophic. The dermal-epidermal junction of the newborn skin is a vital area of attachment. Any defects in the components that comprise this junction can lead to fragility of the skin. EB diagnosis and testing can be per formed both prenatally and postnatally. There is no cure for EB. Treatment revolves around supportive care and prevention of complications. The NICU nurse plays an important role on the multidisciplinary team as primary caregiver to the infant and educator to the family.