Ehlers-Danlos Syndrome is a group of heritable disorders of connective tissue. The major clinical features are hyperextensible skin, hypermobile joints and tissue fragility. There are several types of EDS each with their own specific treatment:
Classical EDS (EDS I & II)
This is the most easily recognised form of Ehlers-Danlos Syndrome. It is characterised by marked skin hyperextensibility and tissue fragility and by joint hypermobility.
Hypermobile EDS (EDS II)
This is both the commonest and the most molecularly heterogeneous EDS subtype, with a population frequency of up to 15%. The main clinical features of this subtype are marked joint hypermobility, moderate skin involvement and no tissue fragility.
Vascular EDS (EDS IV)
This is the most severe form of EDS. The life expectancy of affected persons is significantly reduced due to spontaneous vascular rupture as well as rupture of the internal organs.
Kyphoscoliotic EDS (EDS VI)
In addition to the features of Classical EDS, this condition is characterized by severe muscular hypotonia after birth, progressive kyphoscoliosis, a Marfanoid habitus, and osteopaenia and ruptures of the eye and great arteries in some cases.
Arthrochalasic EDS (EDS VIIA & VIIB)
This type of EDS is characterized by congenital bilateral hip dislocation, severe joint hypermobility, moderate skin involvement and osteopaenia.
Dermatosparactic EDS (EDS VIIC)
Redundant and fragile skin, prominent herniae, joint laxity and dysmorphic features characterize this type of EDS.
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