Assessment of skin extensibility and joint hypermobility in patients with spontaneous cervical artery dissection and Ehlers-Danlos syndrome

Abstract

Ehlers-Danlos syndrome (EDS) is a hereditary connective tissue disorder. One important clinical characteristic of classical type EDS is skin hyperextensibility. Examination of clinical evidence and electron microscopic views of skin biopsies suggest that connective tissue abnormalities resembling very mild EDS are present in a sizable proportion of patients with spontaneous cervical artery dissection (sCAD). Manual assessment of skin extensibility is difficult. Therefore, non-invasive machine-aided measurement of skin extensibility was used and compared with manual assessment of skin extensibility and joint hyperextensibility. Patients with classical EDS, vascular-type EDS, sCAD and healthy patients were evaluated. Skin extensibility was measurably and palpably elevated in all patients with classical type EDS but not in sCAD patients or patients with vascular-type EDS compared to healthy control individuals. Our method is able to measure the increased skin extensibility in classical type EDS. Increased skin extensibility is not present in sCAD patients.