Letter to the Editor
“Benign Hypermobility”-Hyperlax Ehlers–Danlos Syndrome. Other Comorbidities
«Hipermovilidad benigna»-síndrome de Ehlers-Danlos hiperlaxo. Otras comorbilidades asociadas
Ana Isabel Turrión Nievesa,b, Henry Moruno Cruza, Rafael Martín Holguerab, Ana Isabel Sanchez-Atrioa,
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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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Other Comorbidities" "tieneTextoCompleto" => true "saludo" => "Dear Editor," "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "263" "paginaFinal" => "264" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Ana Isabel Turrión Nieves, Henry Moruno Cruz, Rafael Martín Holguera, Ana Isabel Sanchez-Atrio" "autores" => array:4 [ 0 => array:3 [ "nombre" => "Ana Isabel" "apellidos" => "Turrión Nieves" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 1 => array:3 [ "nombre" => "Henry" "apellidos" => "Moruno Cruz" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "Rafael" "apellidos" => "Martín Holguera" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:4 [ "nombre" => "Ana Isabel" "apellidos" => "Sanchez-Atrio" "email" => array:1 [ 0 => "anaturrion@hotmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Enfermedades del Sistema Inmune-Reumatología, Departamento de Medicina y Especialidades Médicas, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Departamento de Cirugía y Ciencias Médico Sociales, Unidad Docente de Anatomía, Universidad de Alcalá de Henares, Alcalá de Henares, Madrid, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "«Hipermovilidad benigna»-síndrome de Ehlers-Danlos hiperlaxo. Otras comorbilidades asociadas" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">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.</p><p id="par0010" class="elsevierStylePara elsevierViewall">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.,<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> a score higher than 4 in Beighton's test<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">2</span></a> is the essential criterion for the diagnosis of this pathology, along with the presence of pain.</p><p id="par0015" class="elsevierStylePara elsevierViewall">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.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">3</span></a> 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 involvement<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">4</span></a> (more or less flexible, silky, etc.).</p><p id="par0020" class="elsevierStylePara elsevierViewall">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.</p><p id="par0025" class="elsevierStylePara elsevierViewall">The most interesting data we have gathered in our hypermobility/Ehlers–Danlos syndrome unit during the last 3 years is the following:<ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">–</span><p id="par0030" class="elsevierStylePara elsevierViewall">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.</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">–</span><p id="par0035" class="elsevierStylePara elsevierViewall">Affectionate-emotional disorder was observed in 14 patients (60%). Of those, 3 had attempted suicide.</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">–</span><p id="par0040" class="elsevierStylePara elsevierViewall">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.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">5,6</span></a> We have not studied how many patients presented fibromyalgia or chronic fatigue syndrome criteria<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">7</span></a> either.</p></li></ul></p><p id="par0045" class="elsevierStylePara elsevierViewall">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” syndrome<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">8</span></a> and rule out the possible presence of a vascular form or type IV, which is associated to visceral aneurysmal malformations.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">4,9</span></a> 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.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">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. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 10 | 15 | 25 |
| 2024 October | 45 | 60 | 105 |
| 2024 September | 77 | 45 | 122 |
| 2024 August | 76 | 60 | 136 |
| 2024 July | 67 | 56 | 123 |
| 2024 June | 67 | 62 | 129 |
| 2024 May | 63 | 60 | 123 |
| 2024 April | 74 | 51 | 125 |
| 2024 March | 79 | 67 | 146 |
| 2024 February | 46 | 42 | 88 |
| 2024 January | 69 | 22 | 91 |
| 2023 December | 58 | 37 | 95 |
| 2023 November | 56 | 74 | 130 |
| 2023 October | 70 | 50 | 120 |
| 2023 September | 87 | 56 | 143 |
| 2023 August | 46 | 34 | 80 |
| 2023 July | 56 | 46 | 102 |
| 2023 June | 44 | 50 | 94 |
| 2023 May | 53 | 49 | 102 |
| 2023 April | 40 | 41 | 81 |
| 2023 March | 46 | 46 | 92 |
| 2023 February | 50 | 43 | 93 |
| 2023 January | 57 | 53 | 110 |
| 2022 December | 49 | 62 | 111 |
| 2022 November | 60 | 40 | 100 |
| 2022 October | 74 | 56 | 130 |
| 2022 September | 59 | 68 | 127 |
| 2022 August | 50 | 57 | 107 |
| 2022 July | 57 | 70 | 127 |
| 2022 June | 40 | 61 | 101 |
| 2022 May | 41 | 51 | 92 |
| 2022 April | 81 | 68 | 149 |
| 2022 March | 106 | 75 | 181 |
| 2022 February | 117 | 56 | 173 |
| 2022 January | 55 | 48 | 103 |
| 2021 December | 37 | 60 | 97 |
| 2021 November | 40 | 49 | 89 |
| 2021 October | 41 | 64 | 105 |
| 2021 September | 43 | 58 | 101 |
| 2021 August | 37 | 69 | 106 |
| 2021 July | 29 | 46 | 75 |
| 2021 June | 39 | 39 | 78 |
| 2021 May | 35 | 33 | 68 |
| 2021 April | 65 | 81 | 146 |
| 2021 March | 94 | 34 | 128 |
| 2021 February | 38 | 28 | 66 |
| 2021 January | 41 | 25 | 66 |
| 2020 December | 35 | 15 | 50 |
| 2020 November | 29 | 21 | 50 |
| 2020 October | 29 | 17 | 46 |
| 2020 September | 29 | 17 | 46 |
| 2020 August | 42 | 22 | 64 |
| 2020 July | 20 | 21 | 41 |
| 2020 June | 33 | 18 | 51 |
| 2020 May | 44 | 13 | 57 |
| 2020 April | 26 | 17 | 43 |
| 2020 March | 25 | 10 | 35 |
| 2019 September | 1 | 0 | 1 |
| 2018 May | 4 | 1 | 5 |
| 2018 April | 41 | 6 | 47 |
| 2018 March | 40 | 9 | 49 |
| 2018 February | 23 | 3 | 26 |
| 2018 January | 13 | 3 | 16 |
| 2017 December | 39 | 7 | 46 |
| 2017 November | 33 | 8 | 41 |
| 2017 October | 36 | 7 | 43 |
| 2017 September | 23 | 2 | 25 |
| 2017 August | 25 | 6 | 31 |
| 2017 July | 27 | 12 | 39 |
| 2017 June | 37 | 10 | 47 |
| 2017 May | 36 | 10 | 46 |
| 2017 April | 35 | 6 | 41 |
| 2017 March | 28 | 6 | 34 |
| 2017 February | 20 | 8 | 28 |
| 2017 January | 35 | 12 | 47 |
| 2016 December | 61 | 13 | 74 |
| 2016 November | 80 | 5 | 85 |
| 2016 October | 85 | 8 | 93 |
| 2016 September | 82 | 4 | 86 |
| 2016 August | 55 | 6 | 61 |
| 2016 July | 30 | 5 | 35 |
| 2015 December | 2 | 0 | 2 |
| 2015 November | 1 | 26 | 27 |
| 2015 October | 4 | 26 | 30 |
| 2015 September | 2 | 0 | 2 |
| 2015 August | 6 | 48 | 54 |
| 2015 July | 36 | 29 | 65 |
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