ABSTRACT

The EDSs are a heterogeneous group of heritable connective tissue disorders characterized by articular hypermobility, skin hyperextensibility and tissue fragility. Six major types are defined according to the Villefranche nosology (1998). For each type, major and minor signs were defined. The presence of one or more major criteria is necessary for clinical diagnosis, while the presence of one or more minor criteria contributes to the diagnosis of a specific type of EDS, but is not sufficient to establish a diagnosis.