A review of the names and terminology used in Epidermolysis Bullosa

Epidermolysis Bullosa (EB) is a group of rare genetic disorders with abnormally fragile skin. There are many different types; some are mild while others can be disabling or even lethal. With advances in science, new types are being recognized all the time. To make things even more confusing, different names have been used over the years for the same type, making it difficult for patients and professionals to understand and learn about these conditions. This international group of EB experts meets about every six years. They review the terminology in light of scientific advances, to make the disease names more meaningful and relevant. This latest version keeps the “onion skin” system which approaches the diagnosis of EB in layers: blister level, clinical features, inheritance pattern and genetic mutation. The traditional EB categories ‐ simplex, junctional, dystrophic and Kindler EB ‐ are kept, with minor changes. In the past, conditions were often named after the doctors who first described them, but these “eponyms” have all been dropped now, except Kindler. Blisters are the hallmark of EB, but conditions where skin fragility shows as peeling, erosions or scaling can also cause serious problems for patients. The new classification has two main EB categories: “Classical EB” and “Other disorders with skin fragility”. The group wanted to keep these “EB‐related” disorders under the EB umbrella, so that all patients with skin fragility can still benefit from treatments for EB. This new EB classification will be useful for patients, health‐care providers and scientists. Linked Article: Has et al. Br J Dermatol 2020; 183 :614–627.