Over the past few decades increasing numbers of health care professionals worldwide have become interested in and actively involved in the day to day care of children and adults with inherited epidermolysis bullosa (EB). Although not too many years ago the care of the EB child was provided primarily by consulting dermatologists and pediatricians, major advances in care during neonatal life and infancy have led to the survival in adulthood of most EB children, even those with the most severe forms. As a result, nearly every medical and allied health practitioner now plays a critical role in the care of these patients, and multidisciplinary care has become the norm. One very visible example of this is the creation of the eb-haus Austria (as discussed in Chapter 3.2), a freestanding, independently funded unit devoted solely to the multidisciplinary management of EB children and adults. It is hoped that similar facilities will be forthcoming elsewhere in the world, so as to best meet the many needs of these patients and their parents.
Within the same timeframe, major advances have been forthcoming which have given great insight into EB at the clinical, epidemiological, cellular, and molecular levels. As the result of nearly 20 years of epidemiological data collection and analysis by the American National EB Registry, for example, we now know for each major EB subtype its prevalence and incidence, the range of cutaneous manifestations, the risk of extracutaneous complications, and its natural history. At the same time, the molecular basis of each major EB subtype has now been elegantly elucidated, setting the stage for gene therapy. Similar advances in our detailed understanding of wound healing should also lead to the development of more effective treatment strategies for our patients, which will be greatly needed until a cure becomes a reality.
