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DIFFERENTIATING EHLERS-DANLOS SYNDROME FROM JOINT HYPERMOBILITY SYNDROME

Ehlers-Danlos Syndrome should be considered in the evaluation of every hypermobile patient who has a pain complaint. A key characteristic for diagnosis and differentiation of EDS from JHS is the addition of skin findings.³⁹ EDS type 1 and type 2, previously known as EDS classic type, has skin that extends easily and snaps back after release. (See Figure 6.) The skin is fragile, as manifested by splitting of the dermis following relatively minor trauma, especially over pressure points (knees, elbows) and areas prone to trauma (shins, forehead, chin). Wound healing is delayed, and the stretching of scars after apparently successful primary wound healing is characteristic. While EDS type 4, vascular type, can have all of the above, besides easy bruising and hematoma formation in areas of trauma, the skin is translucent (visible veins on the chest).⁴⁰ These skin findings are in contrast to EDS type 3, hypermobility type, which has soft skin with normal or only slightly increased extensibility.⁴¹

Figure 6.Patients with EDS type 1 and type 2 demonstrate skin that extends easily and snaps back after release.

Skin hyperextensibility is assessed at a site lacking excess or loose skin
and without evidence of prior trauma by gently pulling until resistance is
met. An ideal location is the volar surface of the forearm, where the upper
limit of normal extensibility is 1-1.5 cm. Extensor surfaces of joints have
excess skin and should not be used.

All forms of EDS, like JHS, affect the joints, causing hypermobility, and as a result, individuals are more susceptible to dislocations, subluxations, sprains, strains, and sometimes fractures. While there is no distinguishing feature of the joint and neuromuscular symptoms of EDS versus JHS, EDS is often more disabling.^{42, 43} The results of one study showed that 1) chronic pain in EDS is highly prevalent and associated with regular use of analgesics; 2) pain is more prevalent and more severe in the hypermobility type; 3) pain severity is correlated with hypermobility, dislocations, and previous surgery; 4) pain is correlated with low nocturnal sleep quality; and 5) pain contributes to functional impairment in daily life, independent of the level of fatigue. The authors concluded, “Therefore, treatment of pain should be a prominent aspect of symptomatic management of EDS.”⁴⁴ In another comprehensive study on EDS, researchers found that over 90% suffered with chronic pain; eight was the mean number of pain locations; 70% reported continuous pain in their lower extremities, ankles, feet, toes, and hips; 89% of the pain began in childhood or adolescence; 88% were or had taken pain medications; and 51% needed narcotics. These authors concluded, “In summary, our data reveals that individuals with EDS experience frequent and severe pain through much of their lives.”⁴⁵ The severe crippling pain of EDS often prevents patients from participating in sports and having a lower quality of life.⁴⁶ Other authors have confirmed the widespread pain symptoms of the condition with the hypermobility type of EDS to be the most debilitating form, with respect to musculoskeletal function, especially affecting ambulatory ability.^{47, 48}