Article - How EDS Got It's Name

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Firstly, 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, ten 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, appeared 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 of report of the syndrome is found, is in Moscow in 1891

where a Dr. A.N. Chernogubov resented 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 abnormality of the connective tissue. The article written by Dr.

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 on to Paris in 1899 where Ehlers from Denmark spoke at a

clinical meeting of the Paris Society of Syphilogy 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 had hyperextensible skin and had developed pigmented

lesions over the bony prominences due to minor traumas.

Again in Paris, about nine years later, Henri-andre 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 patients skin and

stated the lesions over the bony prominences where post-traumatic

'psuedo-tumours' 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 Khon 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 named such

as Cutis Hyperlastica by which it was once known.