|Written by Valerie Burrows |
A syndrome is a collection of physical features, which, when they occur together, enable a physician to recognize a certain condition.
Many different problems occur in Ehlers–Danlos Syndrome (EDS) and not everyone will have all the problems or have them to the same degree but at least some of them must be present for a diagnosis of Ehlers–Danlos Syndrome. There are, at least, nine different types of EDS and the severity, even within the type, can vary in the same family.
The history of EDS begins in 1657 where the first written reports, by a surgeon called Van Meerken, appear in Latin. Although it was noted by Hippocrates in 400 BC that Nomads and Scythians were lax of joint and had multiple scars. Van Meerken describes the hyperelastic skin of a Spanish sailor and the joint laxity of a professional contortionist who Van Meerken presented to a group of senior physicians at the Academy of Leiden.
The next place a report of the syndrome is found is in Moscow in 1891 where a Dr. A.N. Chernogubov presented a boy of seventeen years old at a meeting of the Moscow Venereology and Dermatology Society. The boy had recurrent joint dislocations and cutaneous nodules, his skin was hyperextensible and fragile and he had multiple scars resulting from minor injuries. Chernogubov accurately diagnosed that the clinical manifestations were caused by an abnormality of the connective tissues. The article written by Chernogubov did not come to the notice of Western Europe at that time however, to this day, Ehlers–Danlos Syndrome is still known by his name in his own country.
We now move to Paris in 1899 where Edward Ehlers from Denmark spoke at a clinical meeting of the Paris Society of Syphilology and Dermatology. The patient he presented was a law student from the Island of Bornholm in the Gulf of Bosnia. The young man had joint laxity and orthopaedic complications. He also has hyperextensible skin and had developed pigmented lesions over the bony prominences due to minor traumas.
Again in Paris, about nine years later, Henri-Alexandre Danlos gave a presentation to the same Society. Danlos was specifically interested in Dermatology and his scientific work received recognition in 1904 when he was elected President of the Paris Medical Society then, in 1906, he became Secretary of the Dermatological Society of Paris. At the 1908 presentation, Danlos disagreed with the diagnosis originally made by another physician and drew attention to extensibility and fragility of the patient's skin. He stated that the lesions over the bony prominences where posttraumatic 'pseudo tumors' in a patient with an inherent defect which he termed 'cutis laxa'. There followed a discussion in which the original physician maintained his initial diagnosis but Danlos persisted and drew attention to Ehlers report in 1901 and one from the Berne Congress by Kohn in 1906.
By 1936, other isolated cases had occurred and there was some problem over the name, it was proposed in an article in the British Journal of Dermatology by Frederich Parkes-Weber that it be named Ehlers–Danlos Syndrome and this has now gained universal acceptance over other names such as Cutis Hyperlastica by which it was once known.
by Valerie Burrows
Reprinted with permission from The Ehlers-Danlos Support Group UK