Ehlers-Danlos syndrome--a historical review

Abstract

Ehlers-Danlos syndrome is an inherited heterogeneous group of connective tissue disorders, characterized by abnormal collagen synthesis, affecting skin, ligaments, joints, blood vessels and other organs. It is one of the oldest known causes of bruising and bleeding and was first described by Hipprocrates in 400 BC. Edvard Ehlers, in 1901, recognized the condition as a distinct entity. In 1908, Henri-Alexandre Danlos suggested that skin extensibility and fragility were the cardinal features of the syndrome. In 1998, Beighton published the classification of Ehlers-Danlos syndrome according to the Villefranche nosology. From the 1960s the genetic make up was identified. Management of bleeding problems associated with Ehlers-Danlos has been slow to progress.