ADAMTS2 and Ehlers-Danlos syndrome: Severe progressive cardiac-valvular problems distinguish the cardiac-valvular EDS type (COL1A2) from cEDS, severe skin fragility and unusual craniofacial features discriminates the dermatosparaxis EDS (ADAMTS2), whereas (congenital) kyphoscoliosis and muscle hypotonia differentiates the kyphoscoliotic EDS (PLOD1, FKBP14), which are other rare recessive EDS types.