ADAMTS2 and Ehlers-Danlos syndrome, dermatosparaxis type: Instead, absence of ADAMTS2 activity causes dermatosparaxis EDS presenting with extreme skin fragility, characteristic craniofacial features, redundant skin with excessive skin folds at the wrists and ankles, umbilical hernia and severe bruising with a risk of subcutaneous hematomas and hemorrhages (Colige et al., 1999).