Epidermolysis bullosa (EB) describes a rare complex group of inherited skin fragility disorders. Ideally patients should be managed in a specialist centre. EB is a lifelong disorder that requires specialist intervention and considerations to minimise complications and improve quality of life.
EB can be inherited autosomal recessively or autosomal dominantly; in general, recessive forms tend to be more severe. More than 1,000 recorded mutations in 14 genes contribute to the various forms of EB, resulting in a huge variety of clinical presentations.
These guidelines, developed by leading expert clinicians in EB, outline each main subtype and focus on the different skin and wound management requirements, as well as general principles for wound management for all types of EB.
