Hereditary epidermolysis bullosa (HEB) is a group of genetic disorders characterized by fragile skin and mucous membranes with varying degrees of severity. The overall condition is generally classified into three major entities depending on the location of the bulla formation: (a) EB simplex (HEBS) which develops intraepidermal blisters at the level of the basal and suprabasal epidermal layers; (b) junctional EB (JHEB) developing blisters within the dermoepidermal basement membrane, primarily at the level of lamina lucida; and (c) dystrophic EB (DHEB) in which the blisters are located underneath the basement membrane in the upper papillary dermis. In addition, Kindler syndrome is considered as a particular subtype of HEB. Further classification of the group continues to evolve based on new criteria and recognition of up to 30 clinical subtypes [1, 2] (Table 39.1).
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