Ehlers-Danlos syndrome(EDS)is a hereditary connective tissue disease with genetic heterogeneity,affecting multiple systems throughout the body.The classic EDS(cEDS)is the most common type,mainly attributed to the mutations in collagen type V alpha 1/collagen type V alpha 2(COL5A1/COL5A2).The typical clinical features of cEDS include cutaneous hyperextensibility,atrophic scarring and joint hypermobility.In 1998,EDS was categorized according to the Villefranche nosology,and simultaneously,the diagnostic criteria for EDS were put forward.Currently,there is no standardized treatment protocol for cEDS,and the management of this disease primarily involves treatment,monitoring and prevention of complications.This paper provides a detailed review of the etiology and pathogenesis,clinical manifestations,diagnosis and differential diagnosis,as well as treatment approaches in cEDS based on current research status at home and abroad.
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