As early as 400 BC, Hippocrates recorded in Airs, Waters and Places certain nomadic populations to be lax joint and to have multiple scars (Brazzaventre et al., 2013).
However, the first complete description of EDS features was only given in 1892 by a Russian dermatologist called Alexandr Nicolaevitch Chernogubow. During the first meeting of Moscow Dermatology and Venereology Society, he presented two patients with associated recurrent dislocations, cutaneous nodules, hyperextensible skin, and multiple scars from minor injuries. A. N. Chernogubow accurately diagnosed that the EDS features were caused by an abnormality of the connective tissues (Denko, 1978). He published his findings in an article but it did not come to the notice of Western Europe at that time. The syndrome was consequently called Chernogubov's syndrome in Russia, whereas it was named differently in Western Europe, a few decades later.
The Ehlers-Danlos terminology of the syndrome was given after a Danish dermatologist called Edvard Ehlers, and a French one called Henri-Alexandre Danlos. Both successively presented patients at a clinical meeting of the Paris Society of Syphilology and Dermatology.
The first patient presented in 1899 by Ehlers had a history of late walking and suffered from frequent subluxations of the knees, multiple haematoma from minor trauma, discoloured lesions on the elbows, knees and knuckles. He also had extensible skin and lax digits. Ehlers published in 1901 a case study in which he defended the idea of a distinct condition. In that study he nearly identified all of the symptoms that are known today to be associated with the conditions.
The second patient was presented in 1908 by Danlos after being diagnosed with a skin disease called pseudodiabetic xanthomata, due to bruising on his elbows and knees. Danlos questioned this diagnosis by drawing attention to other features similar to the ones observed in the clinical case presented by Ehlers a few years earlier, namely extensibility and fragility of the skin. He suggested that all of these manifestations originated from a common disturbance that he named “cutis laxa”, i.e. “loose skin”, with skin extensibility and fragility as cardinal features.
Finally, in 1936, an English physician called Frederick Parkes-Weber, suggested that the disorder could be referred as Ehlers-Danlos syndrome.(Parapia et Jackson, 2008 ; Laferrier et al., 2018).
References :
Brazzaventre, C. Celletti, C. Gobattoni, P. Santilli, V., Camerota, F. (2013) ‘The Ehlers-Danlos syndrome: history of a clinical hendiadys’, Medicina nei Secoli, 25(2) pp. 491-501.
Whonamedit (unknown), Ehlers-Danlos Syndrome. Available at http://www.whonamedit.com/synd.cfm/2017.html (Accessed : 18 November 2018)
Denko, C. W. (1978), ‘Chernogubov's syndrome: a translation of the first modern case report of the Ehlers-Danlos syndrome.’, J Rheumatol, 5(3):347-52.
Parapia, L.A. Jackson, C.(2008), ‘Ehlers‐Danlos syndrome – a historical review’, Br J Haematol., 141(1):32-5.
Laferrier, J.Z. Muldowney, K. Muldowney, K. (2018), ‘A Novel Exercise Protocol for Individuals with Ehlers Danlos Syndrome: A Case Report’, J Nov Physiother, 8(2): 382
Recognition of EDS
