Skin extensibility and consistency in patients with Ehlers-Danlos syndrome and benign joint hypermobility syndrome.

OBJECTIVES

The reproducibility of clinical tests for skin extensibility and consistency, essential for differentiating between types of Ehlers-Danlos syndrome (EDS) and benign joint hypermobility syndrome (BJHS), is unknown. Paraclinical methods may provide objective differential diagnostic methods.

METHODS

Six EDS, 11 BJHS, and 19 controls completed the trial. We analysed the overall inter-examiner agreement on clinical tests for skin extensibility and consistency, in addition to analyses on suction cup (SC) and soft tissue stiffness meter (STSM) methods.

RESULTS

Overall agreement on tests for skin extensibility and consistency varied between 0.44 and 0.72. Extensibility evaluated by SC showed an insignificant difference between EDS patients and controls (p = 0.056). Consistency evaluated by STSM showed significant differences (p = 0.001).

CONCLUSIONS

Overall inter-examiner agreement on clinical tests for skin extensibility and consistency was below 0.80, which was required a priori to conduct a reproducibility study. Further refinement of tests and a training phase are necessary. The SC and STSM results are encouraging but must be reproduced in a larger study population.