Brazzaventre Cristina, Celletti Claudia, Gobattoni Paolo, Santilli Valter, Camerota Filippo
Dipartimento di Medicina Fisica e Riabilitativa, Policlinico Umberto I, "La Sapienza" Universitià di Roma, I.
Med Secoli. 2013;25(2):491-501.
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 scarring. 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 chronicfatigue or widespread pain, there should be prompt an appropriate intervention.
埃勒斯-当洛综合征(EDS)是一组临床和遗传异质性的遗传性结缔组织疾病,其特征为关节活动过度、皮肤过度伸展和组织脆弱,导致容易出现瘀伤和异常瘢痕形成。该病症表现出从较轻到严重的表型差异。与活动(活动过度、脱位、平衡受损)、疼痛(全身疼痛、头痛、颌部和牙齿疼痛)和皮肤(瘀伤、脆弱、伤口愈合受损)相关的主诉很常见。公元前400年,希波克拉底在其著作《空气、水和地方》中首次指出,游牧民族斯基泰人有关节松弛和多处瘢痕。虽然额外的灵活性在活动能力和敏捷性方面可能有益,但组织松弛和脆弱的不良影响可能会导致临床后果。我们认识到,对于那些出现慢性疲劳或广泛性疼痛等潜在致残症状的活动过度患者,及时进行适当干预非常重要。