The Ehlers-Danlos Syndrome: History of a Clinical Hendiadys

Cristina Brazzaventre, Claudia Celletti, Paolo Gobattoni, Valter Santilli, Filippo Camerota

Abstract


Ehlers-Danlos syndrome (EDS) is a clinically and genetically heterogeneous group of inherited connective tissue disorders characterized by joint hypermobility, skin hyperextensibility and tissue fragility, which results in easy bruising and abnormal scarring1. The condition shows a phenotypic variance from milder to serious presentations.
Complaints related to activity (hypermobility, dislocations, impaired balance), to pain (general pain, headache, jaw and tooth pain) and to skin (bruises, fragility, impaired wound healing) are frequent.
It was first noted by Hippocrates in 400 BC in his writing Airs Water and Places that the nomads Scythians had lax joints and multiple scars. Whereas the additional flexibility can give benefits in term of mobility and agility, adverse effects of tissue laxity and fragility can give rise to clinical consequences.
We recognize that it is important that, in those hypermobility patients, who develop potentially debilitating symptoms of chronic fatigue or widespread pain, there should be prompt an appropriate intervention.


Key words: Ehlers-Danlos syndrome Hypermobility - Scythians


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