Electron microscopy studies on skin biopsies from affected individuals initially suggested EDS to be a disorder of the collagen ‘wickerwork’ [2], a suspicion that was subsequently confirmed by the biochemical and genetic identification of defects in the primary structure of fibrillar collagens, type I, III and V or their modifying enzymes (lysyl hydroxylase 1 and type I procollagen amino-proteinase) in individuals with different types of EDS [3,4]. This evidence concerns the gene PLOD1 and Ehlers-Danlos syndrome.