Les multiples douleurs du syndrome d'Ehlers-Danlos. Description et proposition d'un protocole thérapeutique

The syndrome described by Ehlers in 1900 still remains unknown to almost all physicians or is only limited to two important signs: skin stretchability and joints hypermobility. The major symptoms causing most of the disability situations among these patients are absent from the classifications established by geneticists in Villefranche in 1997. Therefore, a complete revision of the semiology of the Ehlers Danlos syndrome is required. Besides widespread, intense and permanent pains with very violent outbursts, in contrast with the absence of imagery, a new space must be made to include joints disorders (including together sprains, dislocations, proprioceptive disorders), dystonia, respiratory, digestive, urinary, auditory, sight, and cognitive disorders which seriously hinder the patients social life. Iatrogenic consequences are numerous and dominated by abusive psychiatric placement, erroneous surgery indications and haemorrhagic accidents, which can lead to death for fragile patients. Usual pain therapies are not all recommended or have often unwanted side effects. It is true for morphine, corticoids, anti-depressant and anti-epileptics. It is therefore important to identify correctly these patients and their fragilities to prevent iatrogenic accidents and to implement efficient therapeutics, such as orthesis (in particular special proprioceptive clothes), oxygen therapy and local pain treatments (in particular local injections of lidocaine). Family behaviour is key and linked to a large diffusion of the syndrome as when a parent is diagnosed with the syndrome, all his children will have it, even if at different degree of severity.