Background: Since publication in 2000 of the Second International Consensus Report on Diagnosis and Classification of Epidermolysis Bullosa, many advances have been made to our understanding of this group of diseases, both clinically and molecularly. At the same time, new epidermolysis bullosa (EB) subtypes have been described and similarities with some other diseases have been identified.
Objective: We sought to arrive at a new consensus of the classification of EB subtypes.
Results: We now present a revised classification system that takes into account the new advances, as well as encompassing other inherited diseases that should also be included within the EB spectrum, based on the presence of blistering and mechanical fragility. Current recommendations are made on the use of specific diagnostic tests, with updates on the findings known to occur within each of the major EB subtypes. Electronic links are also provided to informational and laboratory resources of particular benefit to clinicians and their patients.
Limitations: As more becomes known about this disease, future modifications may be needed. The classification system has been designed with sufficient flexibility for these modifications.
Conclusion: This revised classification system should assist clinicians in accurately diagnosing and subclassifying patients with EB.