Investigation into the Ehlers-Danlos syndrome

Abstract

Since the 17th century there has been a certain confusion in diagnosis of what is now called the Ehlers-Danlos syndrome. In 1682 Meekrin described the case of a Spaniard who could make his skin stretch to an enormous extent, and appears to have confused the Ehlers-Danlos syndrome with the condition of cutis laxa. No further significant literature appeared until Unna (1896) described the histology of elastic skin, but the syndrome as such had not then been recognised. In 1900 Ehlers presented a case at the Danish Society of Dermatology which he described as "Cutis laxa, with a tendency to haemorrhage in the skin and to laxity of the joints", and in the same year Sir Malcolm Morris demonstrated a case at the British Dermatological Society which he called "Loose skin with numerous cutaneous nodules". This was followed in 1906 by a case shown by Hallopeau and Lepinay which they called "Diabetic Xanthorum" but which was probably a case of the Ehlers-Danlos syndrome, as was the case described by Cohn (1907) in Germany. The case of Hallopeau and Lepinay was further reviewed in 1908 by Danlos in France, who described it as "Cutis Laxa with Tumours 'par contusion chronique des coudes et des genoux'". The same case was investigated in the same year by Pautrier who was the first to describe the histology of the condition.