C. Hamonet, I Brock
Hypermobility in Ehlers-Danlos syndrome was the first historical finding, and is still deemed by many to be a requirement for diagnosis. It was aptly described by Lauritz-Edvard Ehlers on December 15, 1900, at the Danish Society of Dermatology in Copenhagen. Hypermobility is also described in the clinical report of Henri-Alexandre Danlos and Achille Miget who, as the first, associated Ehlers and Danlos in the same syndrome. Currently, there is some controversy about the role of hypermobility, and on how it may be diagnosed. The discovery of muscle and tendon retraction as a part of this syndrome sheds new light on the diagnostic debate surrounding a genetic syndrome in the absence of biological markers, with as of yet no fully conclusive genetic test available for its identification. Our study included 232 patients (84% women) from 2 to 70 years of age. Diagnosis was made in accordance with the Villefranche geneticists classification criteria. The patients enrolled in the study are of the hypermobile type. We looked for muscle, tendon, and aponeurosis retraction in the knees, ankles, and the soles of feet. In our population of 232 patients, we found a retraction of the hamstrings in 203 individuals (87.5%). Retractions of the triceps surae were found in 90.9% of patients, and retractions of the soles of feet were observed in 95.9% of patients. The impact of retraction on the Beighton palms-on-the-floor test is very great indeed 97.8% of patients who present a retraction of the hamstrings of over 45° cannot perform this maneuver. Hamstring retraction does not, however, affect the test of knee hypermobility (recurvatum). The presence of muscle and tendon retractions in the posterior muscle compartments of the lower limbs and the soles of the feet constitute clinical features of Ehlers-Danlos syndrome. They should be addressed with a view to prevention and treatment, mainly through physical therapy.
