Reduced skin thickness: A new minor diagnostic criterion for the classical and hypermobility types of Ehlers-Danlos syndrome

Background: The diagnosis of Ehlers-Danlos syndrome (EDS) is mainly based on clinical criteria, although in some instances a sound molecular diagnosis is available. Clinical signs can be divided into two categories: one with high diagnostic specificity and the other with low specificity. Despite the fact that reduced skin thickness is one of the dermatological features in patients with EDS, this issue has not been analysed in greater detail. Objectives: To determine skin thickness in patients with the classical and the hypermobility types of EDS. Methods: In 21 patients with classical type of EDS and in nine patients with hypermobility type of EDS, skin thickness was analysed at different body sites by cross-sectional b-mode scans obtained with a 20-MHz ultrasound system. Results: We found a significant decrease in skin thickness in both types of EDS, which was highest at the chest and at the distal part of the lower leg. Conclusions: We propose that the reduced thickness of the dermis as determined by high-resolution 20-MHz ultrasound can be used as a new minor criterion in the diagnosis of the classical and the hypermobility types of EDS.