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Ehlers-Danlos syndrome of the hypermobile type : a multisystemic disorder. Contribution of skin ultra-structure to individual management
Hermanns-Lê T. , Piérard G.E. , Piérard-Franchimont C. , Manicourt D.
Rev Med Liege 2015, 70(5-6),325-330Abstract : Ehlers-Danlos syndrome (EDS) represents a heterogeneous group of disorders of the connective tissue structure. Currently, several types are distinguished following a limited set of clinical signs and genetic mutations. However, there is a lack of specificity of most recognized genetic alterations with the current clinical typing. In addition, the criteria from dermatopathology, ultrastructure and biomechanics are not considered. In addition, the established EDS frontiers are hazardous because a series of anatomo-clinical signs are not considered in the classical EDS concept. The hypermobile type EDS represents an example of the diagnostic uncertainties. It results that guidelines based on evidence-based medicine cannot be established. Only an individual management can be offered to the concerned patients.
