Pain in hypermobile Ehlers_Danlos syndrome- New insights using new criteria

K BŽnistan, V Martinez

UniversitŽ Versailles Saint-Quentin, Montigny-Le-Bretonneux, France.

American Journal of Medical Genetics Part A

Features of the pain in hypermobile Ehlers_Danlos syndrome (hEDS) are complex and insufficiently known by clinicians. We enrolled 37 hEDS patients. Disease status was ascertained using revised 2017 International Classification criteria, in the EDS French National Reference Center. Patients were evaluated with a clinical examination, quantitative sensory testing, and validated questionnaires. Thirty_seven patients were evaluated. Pain had appeared at 10_±_5 years old and became chronic at 20_±_9 years old. hEDS was diagnosed at only 24_±_10 years old. Ninety_seven percent of them had severe chronic pain, which gradually increased over time in 75% of them. The main location of pain was in joints and predominated in lower limbs. Patients with a generalized presentation of pain had older chronic pain and a higher impact on the affective component. Neuropathic pain was frequent in the most painful joint and associated with heat hypoesthesia. An asymmetric proprioception was found in one third of the patients. A very high rate of attempted suicide was observed. To conclude, pain in hEDS is severe, chronic, and disabling. Sensorial and proprioceptive sensibilities are also affected. Peripheral neuropathic pain is frequent and central sensitization appears to be a key step in the evolution of disease.

BIOSEB Instruments Used:
Von Frey Filaments (Bio-VF-M)

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