Nutritional Management of the Epidermolysis Bullosa Patient

Abstract

Some degree of nutritional compromise is present in virtually all patients with epidermolysis bullosa (EB). As with all clinical manifestations of EB, the extent of nutritional compromise is determined by the type of EB. It tends to be mild in EB simplex, but can be very severe in junctional and dystrophic forms. In patients with the Hallopeau-Siemens type of recessive dystrophic EB, the combination of esophageal stricture, abnormal dentition, microstomia, and crippling hand deformities results in a picture of severe, recalcitrant nutritional deprivation unparalleled in all of clinical medicine. In this chapter we review the complex etiologies leading to nutritional compromise in EB patients, and discuss management guidelines.