“…The latter also has distinctive tonofilament clumping in basal cells (Anton-Lamprecht and Schnyder, 1982). Junctional and Dystrophic EB are characterized by blistering in the plane of the lamina lucida of the basement membrane zone and below the dermal-epidermal basement membrane at the level of the anchoring fibrils, respectively (Eady, 1988;Fine et al, 1991;Uitto and Christiano, 1992 (laminins) in patients displaying junctional forms of EB (Pulkkinen et al, 1994a,b), whereas mutations within the type VII collagen (COL7Al) gene have been identified in pedigrees displaying dystrophic forms of EB (Christian0 et al, 1993(Christian0 et al, , 1994Hilal et al, 1993;Hovnanian et al, 1994 keratins are numbered K1-K8 and are larger than the type I keratins (numbered K9-K20), owing to their V1 and V2 subdomains, which vary in length and sequence and have a high glycine content (Moll et al, 1982 fashion, to form a heterodimer. Subsequently, two heterodimers form a tetramer and groups of tetramers form protofilaments (2-3 nm), which in turn group to form protofibrils (4-5 nm).…”