The hypermobility type of Ehlers-Danlos syndrome (EDS Type III) features generalized joint hypermobility without some of the skin findings seen in the classical type.


Inheritance: autosomal dominant


Major criteria:

(1) variable skin involvement (hyperextensible skin and/or smooth, velvety skin)

(2) generalized joint hypermobility involving small and large joints


Minor criteria:

(1) recurring joint dislocations

(2) chronic joint or limb pain: Musculoskeletal pain shows an early onset and may be disabling

(3) positive family history


Other findings:

(1) dilatation and/or rupture of the ascending aorta

(2) scoliosis, pes planus and other musculoskeletal changes may be found.


Distinction from the classical type:

(1) Patients with the hypermobility type have normal scarring. The presence of atrophic scars suggests the classical type.

(2) Molluscoid pseudotumors are absent in the hypermobility type.


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