A Survey of Epidermolysis Bullosa Care in the United States and Canada

Abstract

Epidermolysis bullosa (EB) is a group of rare, inherited, blistering diseases that typically present in infancy. EB is not curable, and treatment is entirely supportive. There is a paucity of standardized recommendations to guide management. To assess the current state of EB care, an original online survey was conducted targeting attending physicians experienced with the care of EB. Members of the Society for Pediatric Dermatology residing in the United States and Canada served as the source pool. Parameters assessed included clinic visits, availability of subspecialists, and performance of surveillance studies. Fifty-six completed surveys were analyzed. Most providers saw between 1 and 10 individuals with EB per year in a general dermatology clinic. For each EB type there was considerable variation in the frequency of clinic visits, availability and use of specialists, and use of laboratory and imaging studies. Some agreement was observed in the frequency of follow-up for infants with more severe EB types, as well as for the components of a history, physical, and routine laboratory studies. These findings describe variations in the current state of EB care that pediatric dermatologists provide. The development and acceptance of evidence-based guidelines and outcome measures may lead to greater uniformity in EB care.