Le syndrome d'Ehlers-Danlos de type III. De la physiopathologie à la thérapeutique

Since 17 years, we follow a cohort of patients with Ehlers-Danlos disease type III in concordance with the criteria of Brighton (Grahame) and Villefranche (Beighton). They are now 2350. The observed clinical manifestations, more severe in females, are the expression of a syndrome of tissue fragility and proprioceptive disorders. Hereditary non-mendelian inheritance affect the tissue collagen impairs the strength and responsiveness of the entire connective system of the human body. From this observation result of the pathophysiological consequences to better interpret clinical disease that sixth sense: that of perception of her own body and guide treatment. These aim to restore proprioception, especially using orthosis and particularly compression garments. Oxygen therapy appears to be the best treatment for fatigue. Surgery has very few indications and can cause significant aggravations. The contribution of psychology is important.