Summary

Pains are among the "masters symptoms" that contribute to a strictly clinical diagnosis in the absence of identification of the gene. They are also, with fatigue, a major cause of functional limitations and handicaps. However, the widespread ignorance of the syndrome causes many doctors to ignore it and to reject the linking of pain to the syndrome. This leads to many diagnostic errors and in particular to abusive "psychiatrisations". The painful symptoms are nevertheless very suggestive: diffuse, variable with acute phases, refractory to analgesics general, influenced by the movement, trauma and climate.

The descriptions of the Ehlers-Danlos syndrome have considerably evolved in the last years (1). Beyond the frequent but inconstant hyper mobility and the discreet or absent extendibility, the semiology has become richer. It is now dominated by pains, tiredness, proprioceptive disorders, hemorrhagic syndrome, dysautonomia, dystonia, and a number of digestion, vesico-sphincteral, respiratory, odontological, ENT, ophtalmological, gyneco-obstetrical, cognitive disorders.

Pain is among the most disabling symptoms for these patients together with tiredness and proprioceptive disorders.

Pain is felt diffusely (2). The whole body is described as painful, sometimes since early childhood, and felt mainly in joints (98%) in shoulders, wrist, fingers, elbows, ankle and foot, but also at the in the neck with front headeaches and backpains (including pelvis). They can be permanent and increased by postures (seated or standing) triggering the endless need to change position. They are clearly augmented by motion, sometime with a delay in relation to its start and with a persisting or remaining pain, a kind of debt paid after an activity, which can last several hours or days. Tendons are particularly affected, as well as muscles (82%). Pains can affect the skin, which can also be hit by hyperesthesia (39%) and tolerate badly contacts, including from clothes. Abdomen pains are present in 77% of the cases, they can be violent (like a blade) and very short, pseudo surgery like or more extended and lasting. Thoracic pains (71%) are mostly located in the sternocostal area and can wrongly look like heart pain especially if located on the left side.Pelvic pains happen with menstruations (75%) and are described as particularly intense. Headaches with associated visual disorders are frequent (84%) and described as intense and disabling.These pains happen through violent episodes (reacing close to 10 on analgesical scales) and lasting. They are resisting to usual antalgic treatments from anti pains centers with secondary effects quite visible, except for Tramadol and Acupan.

However, they are sensitive to local treatments (Heat, hydrotherapy, TENS, Versatis, anti inflammatory gels, anesthetic local injections under the skin, while tooth anesthesia, anesthetic blocs and rachis anesthetic are often ineffective). Orthesis (sole, back belsts, compressing proprioceptive clothes, collars, kneepad…) have a direct antalgical effect (TENS effect) and indirect effect (improvement of the biomechanical constraints).

References

(1) C. Hamonet, P. Ravaud, S. Villeneuve, A. Gompel, Serre, D. Fredy, D. Deparcy, G. Mazaltarine, J.D. Zeitoun, A. Metlaine, D. Léger, A. Benachi, A.G. Cordier, P. Césaro, C. Loche, N. Dantschev, M. Vienne, C. Séjourné, N. Laouar, B. Gogly, MM Landru, S. Moussa Badran, Y. Raffray, G. Challe, L. Doursounian, J. Mohler. Ehlers-Danlos (à propos de 664 cas). "Ehlers-Danlos syndrome (about 664 cases). Statistical analysis of clinical signs from 644 patients with a Beighton scale ≥ 4/9". Poster, First international Symposium on the Ehlers-Danlos syndrome, September 8th-11th 2012, Ghent, Belgium.

(2) C. Hamonet, "Les douleurs dans le syndrome d’Ehlers-Danlos (SED)". Colloque Douleurs et maladies rares, Université Paris-Est-Créteil (UPEC), Téléthon, Fondation de France. Campus de Fontainebleau, 8 décembre 2012.

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