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Patients were evaluated with a clinical examination, quantitative sensory testing, and validated questionnaires. Thirty\u2010seven patients were evaluated. Pain had appeared at 10\u2009\u00b1\u20095 years old and became chronic at 20\u2009\u00b1\u20099 years old. hEDS was diagnosed at only 24\u2009\u00b1\u200910 years old. Ninety\u2010seven 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.<\/jats:p>","DOI":"10.1002\/ajmg.a.61175","type":"journal-article","created":{"date-parts":[[2019,5,10]],"date-time":"2019-05-10T16:04:33Z","timestamp":1557504273000},"page":"1226-1234","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":51,"title":["Pain in hypermobile Ehlers\u2010Danlos syndrome: New insights using new criteria"],"prefix":"10.1002","volume":"179","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2193-457X","authenticated-orcid":false,"given":"Karelle","family":"B\u00e9nistan","sequence":"first","affiliation":[{"name":"Centre de r\u00e9f\u00e9rence des syndromes d'Ehlers\u2010Danlos non vasculaires H\u00f4pital Raymond Poincar\u00e9, Garches, Assistance Publique H\u00f4pitaux de Paris  Garches France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valeria","family":"Martinez","sequence":"additional","affiliation":[{"name":"D\u00e9partement d'anesth\u00e9sie et consultation douleur H\u00f4pital Raymond Poincar\u00e9, Garches, Assistance Publique H\u00f4pitaux de Paris  Garches France"},{"name":"Centre d'Evaluation et de Traitement de la Douleur, F\u201092100 France INSERM, U\u2010987, H\u00f4pital Ambroise Par\u00e9  Boulogne\u2010Billancourt France"},{"name":"Universit\u00e9 Versailles Saint\u2010Quentin  Montigny\u2010Le\u2010Bretonneux France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,5,10]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pain.2004.12.010"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pain.2003.12.024"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/0304-3959(92)90112-O"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpainsymman.2010.09.012"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.35483"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.36315"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.c.31539"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000002847"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.34330"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jbspin.2015.04.002"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00221-013-3656-4"},{"issue":"2","key":"e_1_2_8_13_1","first-page":"129","article-title":"Pain assessment: Global use of the brief pain inventory","volume":"23","author":"Cleeland C. 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