Given the reported importance of LH3 in deposition and organization of extracellular matrix [27] and that LH3 modifies proteins in the extracellular space [24], reduction of LH3 likely contributes to the altered dermal microenvironment composition observed in RDEB and in response to type VII collagen depletion in normal fibroblasts [28,29,33]. This evidence concerns the gene PLOD3 and recessive dystrophic epidermolysis bullosa.