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Vol. 11. Issue 4.
Pages 263-264 (July - August 2015)
Vol. 11. Issue 4.
Pages 263-264 (July - August 2015)
Letter to the Editor
DOI: 10.1016/j.reumae.2015.01.007
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“Benign Hypermobility”-Hyperlax Ehlers–Danlos Syndrome. Other Comorbidities
«Hipermovilidad benigna»-síndrome de Ehlers-Danlos hiperlaxo. Otras comorbilidades asociadas
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Ana Isabel Turrión Nievesa,b, Henry Moruno Cruza, Rafael Martín Holguerab, Ana Isabel Sanchez-Atrioa,
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anaturrion@hotmail.com

Corresponding author.
a Servicio de Enfermedades del Sistema Inmune-Reumatología, Departamento de Medicina y Especialidades Médicas, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, Spain
b Departamento de Cirugía y Ciencias Médico Sociales, Unidad Docente de Anatomía, Universidad de Alcalá de Henares, Alcalá de Henares, Madrid, Spain
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Dear Editor,

Nowadays, there are 11 different types of Ehlers–Danlos syndrome described. Types I and II (classic Ehlers–Danlos) and mainly type III are classified as benign hypermobility.

When studying this syndrome, we noticed that the word “hiperlaxitud” (hypermobility) does not exist in the dictionary of the Real Academia de la Lengua (Royal Spanish Academy). The concept of “hypermobility” refers to the articular hypermobility observed in these patients, causing painful symptomatology. As shown in the recent article of Pantoja et al.,1 a score higher than 4 in Beighton's test2 is the essential criterion for the diagnosis of this pathology, along with the presence of pain.

In the first International Symposium on Ehlers–Danlos syndrome, which took place in Ghent in September, 2012, an attempt was made to establish new diagnostic criteria that would offer higher specificity than the old criteria of Brighton and Villefranche.3 These new criteria must allow for the inclusion of truly hiperlax forms and the obtainment of universally accepted instruments for the measurement of laxity, not only articular but also from other tissues, avoiding professional subjectivity in the assessment of mainly cutaneous involvement4 (more or less flexible, silky, etc.).

The first inclusion criterion to be able to diagnose a patient of this pathology is that the patient is over 16, because, physiologically, tissue laxity is greater during childhood. Furthermore, it is important to point out that, physiologically, tissue laxity is higher in women than in men. This pathology usually affects women, as occurs among main connective tissue diseases.

The most interesting data we have gathered in our hypermobility/Ehlers–Danlos syndrome unit during the last 3 years is the following:

  • Out of 23 patients diagnosed with articular laxity, 3 of them were men and 20 were women. The average age of our patients was 33 years. Out of the 23 patients, 18 (78%) were subjected to at least one musculoskeletal surgical intervention or due to peripheral nerve entrapment. Of those 18 patients, 11 needed reintervention of the same pathological process. The surgical interventions average was 3 per patient, and there was one specific case of a patient who was subjected to 13.

  • Affectionate-emotional disorder was observed in 14 patients (60%). Of those, 3 had attempted suicide.

  • In 5 patients (22%), low tension figures were observed and/or there were episodes of vasovagal syncope, in relation to the dysautonomia. In 6 patients (26%) a clear Raynaud's phenomenon with its 3 phases was observed. Autoinmune diseases were discarded for all of them. We observed livedo reticularis in 2 patients. Vitamin D3 deficit was identified in 17 patients (74%). We have not performed densitometric studies on our patients; therefore, we cannot provide data about the potential greater incidence of osteoporosis in subjects with this pathology, as pointed out in studies.5,6 We have not studied how many patients presented fibromyalgia or chronic fatigue syndrome criteria7 either.

As well-indicated in the section “Clinical Rheumatology in Images”, it is of interest to rheumatologists and other experts who assess musculoskeletal pathology to know the necessary information in order to diagnose “benign hypermobility” syndrome8 and rule out the possible presence of a vascular form or type IV, which is associated to visceral aneurysmal malformations.4,9 This vascular type is rare and difficult to diagnose and requires confirmation through genetic tests. Likewise, it is important to know the presence of comorbidities and the high rate of failed surgical interventions that are observed in patients with benign hypermobility, aspects to be taken into account in case of requiring management in surgical units.

References
[1]
L. Pantoja, C. Diez, D. Alexis.
Hyperlax Ehlers–Danlos syndrome: benign hypermobility?.
Reumatol Clin, 10 (2014), pp. 189-190
[2]
P. Beighton, A. de Paepe, B. Steinmann, P. Tsipouras, R.J. Wenstrup.
Ehlers–Danlos syndromes: Revised nosology, Villefranche, 1997. Ehlers–Danlos National Foundation (USA) and Ehlers–Danlos Support Group (UK).
Am J Med Genet, 77 (1998), pp. 31-37
[3]
J.F. Bravo.
Ehlers–Danlos syndrome, with special emphasis in the joint hypermobility syndrome.
Rev Med Chil, 137 (2009), pp. 1488-1497
[4]
J.F. Bravo, C. Wolff.
Clinical study of hereditary disorders of connective tissues in a Chilean population: joint hypermobility syndrome and vascular Ehlers–Danlos syndrome.
Arthritis Rheum, 54 (2006), pp. 515-523
[5]
J.L. Yen, S.P. Lin, M.R. Chen, D.M. Niu.
Clinical features of Ehlers–Danlos syndrome.
J Formos Med Assoc, 105 (2006), pp. 475-480
[6]
S. Gulbahar, E. Sahin, M. Baydar, C. Bircan, R. Kizil, M. Manisali, et al.
Hypermobility syndrome increases the risk for low bone mass.
Clin Rheumatol, 25 (2006), pp. 511-514
[7]
D. Ofluoglu, O.H. Gunduz, E. Kul-Panza, Z. Guven.
Hypermobility in women with fibromyalgia syndrome.
Clin Rheumatol, 25 (2006), pp. 291-293
[8]
I. De Wandele, L. Rombaut, L. Leybaert, P. van de Borne, T. de Backer, F. Malfait, et al.
Dysautonomia and its underlying mechanisms in the hypermobility type of Ehlers–Danlos syndrome.
Semin Arthritis Rheum, 44 (2014), pp. 93-100
[9]
M. Pepin, U. Schwarze, A. Superti-Furga, P.H. Byers.
Clinical and genetic features of Ehlers–Danlos syndrome type IV, the vascular type.
N Engl J Med, 342 (2000), pp. 673-680

Please cite this article as: Turrión Nieves AI, Moruno Cruz H, Martín Holguera R, Sanchez-Atrio AI. «Hipermovilidad benigna»-síndrome de Ehlers Danlos hiperlaxo. Otras comorbilidades asociadas. Reumatol Clin. 2015;11:263–264.

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