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Definition

A severe form of Ehlers-Danlos syndrome, a group of connective tissue disorders characterized by skin hyperextensibility, articular hypermobility, and tissue fragility. EDSVASC is an autosomal dominant disease characterized by joint and dermal manifestations as in other forms of the syndrome, and by proneness to spontaneous rupture of bowel and large arteries. The vascular complications may affect all anatomical areas.

Acronym

EDSVASC

Synonyms

EDS4
EDS IV
Ehlers-Danlos syndrome, type IV, autosomal dominant
Ehlers-Danlos syndrome 4
Ehlers-Danlos syndrome arterial type
Ehlers-Danlos syndrome ecchymotic type
Sack-Barabas syndrome

Keywords

Ehlers-Danlos syndrome

 
Disclaimer

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