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Physical examination showed weakness on the Daniels scale as follows: cervical 2/5, shoulder girdle 3/5, pelvic girdle 2/5, macroglossia, with oedema of the face and limbs (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). Serous creatinine phosphokinase was at 600<span class="elsevierStyleHsp" style=""></span>U/L (normal 38–174<span class="elsevierStyleHsp" style=""></span>U/L), lactate dehydrogenase at 1278<span class="elsevierStyleHsp" style=""></span>U/L (normal 120–300<span class="elsevierStyleHsp" style=""></span>U/L), ANA negative, AST 88<span class="elsevierStyleHsp" style=""></span>U/L normal (10–35<span class="elsevierStyleHsp" style=""></span>U/L), ALT 31<span class="elsevierStyleHsp" style=""></span>U/L (normal 7–35<span class="elsevierStyleHsp" style=""></span>U/L), albumen at 4.2<span class="elsevierStyleHsp" style=""></span>g/dL (normal 3.4–4.8<span class="elsevierStyleHsp" style=""></span>g/dL) and urine test normal. Muscle biopsy was compatible with myositis (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). Electromyography with myopathic pattern. Upper GI series with impaired swallowing. A final diagnosis of juvenile dermatomyositis (JDM) was made. Treatment was started with 5 methylpredinisolone pulse therapy (30<span class="elsevierStyleHsp" style=""></span>mg/kg/day), and subsequently with 1<span class="elsevierStyleHsp" style=""></span>mg/kg/day of prednisone, IV immunoglobulin (2<span class="elsevierStyleHsp" style=""></span>g/kg/total dose) and 11 doses of cyclophosphamide monthly (1<span class="elsevierStyleHsp" style=""></span>g/m<span class="elsevierStyleSup">2</span> sc), with a gradual lowering of the prednisone dose to 5<span class="elsevierStyleHsp" style=""></span>mg/day. When cyclophosphamide was withdrawn treatment with methotrexate was started (15<span class="elsevierStyleHsp" style=""></span>mg weekly), folic acid and chloroquine. The patient was monitored for 3 years and did not present with any further relapses.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">JDM is a generalised inflammatory systemic disease associated with pathognomonic changes to the skin. Subcutaneous oedema as presentation of JDM has been rarely reported and involves an aggressive disease course. Excessive vascular permeability in the muscle and subcutaneous tissues as a result of immune complex deposits, complement activation and damage to the vascular endothelium which leads to muscle microinfarctions may contribute to the presence of oedema.<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">1–3</span></a> Severe cases of JDM are associated with the early onset of subcutaneous oedema in limbs, related to inflammatory vasculopathy with a narrowing or complete occlusion of the lumen of medium-sized and small arteries.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">3</span></a> The absence of nephrotic syndrome, cardiac arrest, hypothyroidism, liver disease and malignancy strongly suggest that the oedema is related to JDM. Whilst reviewing generalised oedema-associated cases of JDM we found 19 cases and it was associated with the presence of severe muscle weakness, gastrointestinal or cutaneous ulcers and a poor response to steroids.<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">2,4</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">In JDM subcutaneous oedema is an indicator of severe disease, and its presence should be a guide for more intensive treatment so as to achieve rapid and appropriate control of the condition.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Ethical Liabilities</span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Protection of people and animals</span><p id="par0020" class="elsevierStylePara elsevierViewall">The authors declare that the procedures followed comply with the ethical standards of the Committee responsible for human experimentation, the World Medical Association and the Declaration of Helsinki.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Data confidentiality</span><p id="par0025" class="elsevierStylePara elsevierViewall">The authors declare that they have adhered to the protocols of their centre of work on the publication of patient data.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Right to privacy and informed consent</span><p id="par0030" class="elsevierStylePara elsevierViewall">The authors have obtained the informed consent of the patients and/or subjects referred to in this article. This document remains in the possession of the corresponding author.</p></span></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Conflict of Interests</span><p id="par0035" class="elsevierStylePara elsevierViewall">The authors have no conflict of interests to declare.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:3 [ 0 => array:3 [ "identificador" => "sec0005" "titulo" => "Ethical Liabilities" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0010" "titulo" => "Protection of people and animals" ] 1 => array:2 [ "identificador" => "sec0015" "titulo" => "Data confidentiality" ] 2 => array:2 [ "identificador" => "sec0020" "titulo" => "Right to privacy and informed consent" ] ] ] 1 => array:2 [ "identificador" => "sec0025" "titulo" => "Conflict of Interests" ] 2 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2017-04-12" "fechaAceptado" => "2017-06-05" "NotaPie" => array:2 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Torres Jiménez AR, Solís-Vallejo E, Céspedes-Cruz AI, Sánchez-Uribe M. Edema subcutáneo en dermatomiositis juvenil. Reumatol Clin. 2019;15:e49–e50.</p>" ] 1 => array:2 [ "etiqueta" => "☆☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0065">The person who wrote the manuscript is Alfonso Ragnar Torres Jiménez.</p>" ] ] "multimedia" => array:2 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 2087 "Ancho" => 2501 "Tamanyo" => 709595 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(A) Macroglossia and facial swelling. (B) Oedema of the hand. (C and D) Facial and hand oedema, 15 days after initiation of treatment.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1225 "Ancho" => 1633 "Tamanyo" => 363494 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Muscle biopsy which shows endomysial lymphocytary infiltrate and myocyte necrosis.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:4 [ 0 => array:3 [ "identificador" => "bib0025" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Increasing recognition of dermatomyositis with subcutaneous edema – is this a poorer prognostic marker?" 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Year/Month | Html | Total | |
---|---|---|---|
2024 October | 21 | 15 | 36 |
2024 September | 72 | 37 | 109 |
2024 August | 82 | 54 | 136 |
2024 July | 60 | 40 | 100 |
2024 June | 73 | 41 | 114 |
2024 May | 55 | 39 | 94 |
2024 April | 54 | 24 | 78 |
2024 March | 57 | 32 | 89 |
2024 February | 61 | 21 | 82 |
2024 January | 87 | 18 | 105 |
2023 December | 57 | 28 | 85 |
2023 November | 67 | 31 | 98 |
2023 October | 59 | 27 | 86 |
2023 September | 96 | 46 | 142 |
2023 August | 85 | 15 | 100 |
2023 July | 49 | 23 | 72 |
2023 June | 57 | 26 | 83 |
2023 May | 65 | 23 | 88 |
2023 April | 52 | 14 | 66 |
2023 March | 75 | 27 | 102 |
2023 February | 66 | 21 | 87 |
2023 January | 71 | 12 | 83 |
2022 December | 71 | 24 | 95 |
2022 November | 55 | 24 | 79 |
2022 October | 73 | 23 | 96 |
2022 September | 48 | 33 | 81 |
2022 August | 36 | 39 | 75 |
2022 July | 54 | 35 | 89 |
2022 June | 33 | 22 | 55 |
2022 May | 47 | 34 | 81 |
2022 April | 48 | 30 | 78 |
2022 March | 47 | 39 | 86 |
2022 February | 57 | 26 | 83 |
2022 January | 77 | 32 | 109 |
2021 December | 28 | 34 | 62 |
2021 November | 46 | 45 | 91 |
2021 October | 54 | 54 | 108 |
2021 September | 43 | 37 | 80 |
2021 August | 28 | 40 | 68 |
2021 July | 27 | 29 | 56 |
2021 June | 41 | 33 | 74 |
2021 May | 37 | 47 | 84 |
2021 April | 113 | 71 | 184 |
2021 March | 50 | 31 | 81 |
2021 February | 39 | 22 | 61 |
2021 January | 29 | 18 | 47 |
2020 December | 38 | 15 | 53 |
2020 November | 22 | 21 | 43 |
2020 October | 25 | 16 | 41 |
2020 September | 42 | 31 | 73 |
2020 August | 22 | 25 | 47 |
2020 July | 24 | 17 | 41 |
2020 June | 41 | 8 | 49 |
2020 May | 34 | 16 | 50 |
2020 April | 27 | 21 | 48 |
2020 March | 8 | 3 | 11 |
2019 September | 1 | 0 | 1 |
2018 December | 1 | 2 | 3 |
2018 November | 0 | 1 | 1 |