ADAMTS2 and Ehlers-Danlos syndrome: ADAMTS2 haploinsufficiency is associated with the dermatosparaxis type of Ehlers–Danlos syndrome (EDS; OMIM 225410) which is inherited in an autosomal recessive manner.[23–25] EDS is a heterogeneous group of disorders that affect the fragility of soft connective tissues, leading to hypermobile joints and hyperextension of the skin and other organs and tissues.