Patients with inherited EB are classified and subclassified on the basis of where the blisters arise within the skin, coupled with their genetic mode of transmission and their clinical phenotype^[2,24] (Tables 33.1 & 33.2). Cutaneous findings play an important role in the overall determination of EB phenotype, even though it is clear that some overlap in the frequency of such findings may occur when too narrow a set of criteria is employed. At present, specific molecular findings are not included as diagnostic tools within an internationally accepted classification system^[2].

All forms of inherited EB are characterized by the presence of mechanically fragile skin, tense fluid-filled blisters, erosions and crusts (Fig. 33.2). Minimal lateral or rotary traction on the skin results in its shearing within ultrastructurally uniform cleavage planes; within minutes, blisters develop in these sites. These changes can be readily induced in most patients, …