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and its presence should be a guide for more intensive treatment so as to achieve rapid and appropriate control of the condition&#46;</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&#44; the World Medical Association and the Declaration of Helsinki&#46;</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&#46;</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&#47;or subjects referred to in this article&#46; This document remains in the possession of the corresponding author&#46;</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&#46;</p></span></span>"
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Images in Clinical Rheumatology
Subcutaneous Edema in Juvenile Dermatomyositis
Edema subcutáneo en dermatomiositis juvenil
Alfonso Ragnar Torres Jiméneza,
Corresponding author
dr-poncho@hotmail.com

Corresponding author.
, Eunice Solís-Vallejoa, Adriana Ivonne Céspedes-Cruza, Magdalena Sánchez-Uribeb
a Reumatología Pediátrica, Hospital General, Centro Médico Nacional La Raza, IMSS, Mexico City, Mexico
b Anatomía Patológica, Hospital de Especialidades, Centro Médico Nacional La Raza, IMSS, Mexico City, Mexico
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We present the case of a 13-year-old female patient with a condition of 15 days onset&#44; unable to get up and to climb up stairs&#44; who presented with myalgias&#44; dysphagia to solids&#44; and oedema of the face and arms&#46; Physical examination showed weakness on the Daniels scale as follows&#58; cervical 2&#47;5&#44; shoulder girdle 3&#47;5&#44; pelvic girdle 2&#47;5&#44; macroglossia&#44; with oedema of the face and limbs &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Serous creatinine phosphokinase was at 600<span class="elsevierStyleHsp" style=""></span>U&#47;L &#40;normal 38&#8211;174<span class="elsevierStyleHsp" style=""></span>U&#47;L&#41;&#44; lactate dehydrogenase at 1278<span class="elsevierStyleHsp" style=""></span>U&#47;L &#40;normal 120&#8211;300<span class="elsevierStyleHsp" style=""></span>U&#47;L&#41;&#44; ANA negative&#44; AST 88<span class="elsevierStyleHsp" style=""></span>U&#47;L normal &#40;10&#8211;35<span class="elsevierStyleHsp" style=""></span>U&#47;L&#41;&#44; ALT 31<span class="elsevierStyleHsp" style=""></span>U&#47;L &#40;normal 7&#8211;35<span class="elsevierStyleHsp" style=""></span>U&#47;L&#41;&#44; albumen at 4&#46;2<span class="elsevierStyleHsp" style=""></span>g&#47;dL &#40;normal 3&#46;4&#8211;4&#46;8<span class="elsevierStyleHsp" style=""></span>g&#47;dL&#41; and urine test normal&#46; Muscle biopsy was compatible with myositis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Electromyography with myopathic pattern&#46; Upper GI series with impaired swallowing&#46; A final diagnosis of juvenile dermatomyositis &#40;JDM&#41; was made&#46; Treatment was started with 5 methylpredinisolone pulse therapy &#40;30<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#47;day&#41;&#44; and subsequently with 1<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#47;day of prednisone&#44; IV immunoglobulin &#40;2<span class="elsevierStyleHsp" style=""></span>g&#47;kg&#47;total dose&#41; and 11 doses of cyclophosphamide monthly &#40;1<span class="elsevierStyleHsp" style=""></span>g&#47;m<span class="elsevierStyleSup">2</span> sc&#41;&#44; with a gradual lowering of the prednisone dose to 5<span class="elsevierStyleHsp" style=""></span>mg&#47;day&#46; When cyclophosphamide was withdrawn treatment with methotrexate was started &#40;15<span class="elsevierStyleHsp" style=""></span>mg weekly&#41;&#44; folic acid and chloroquine&#46; The patient was monitored for 3 years and did not present with any further relapses&#46;</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&#46; Subcutaneous oedema as presentation of JDM has been rarely reported and involves an aggressive disease course&#46; Excessive vascular permeability in the muscle and subcutaneous tissues as a result of immune complex deposits&#44; complement activation and damage to the vascular endothelium which leads to muscle microinfarctions may contribute to the presence of oedema&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">1&#8211;3</span></a> Severe cases of JDM are associated with the early onset of subcutaneous oedema in limbs&#44; related to inflammatory vasculopathy with a narrowing or complete occlusion of the lumen of medium-sized and small arteries&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">3</span></a> The absence of nephrotic syndrome&#44; cardiac arrest&#44; hypothyroidism&#44; liver disease and malignancy strongly suggest that the oedema is related to JDM&#46; Whilst reviewing generalised oedema-associated cases of JDM we found 19 cases and it was associated with the presence of severe muscle weakness&#44; gastrointestinal or cutaneous ulcers and a poor response to steroids&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">2&#44;4</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">In JDM subcutaneous oedema is an indicator of severe disease&#44; and its presence should be a guide for more intensive treatment so as to achieve rapid and appropriate control of the condition&#46;</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&#44; the World Medical Association and the Declaration of Helsinki&#46;</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&#46;</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&#47;or subjects referred to in this article&#46; This document remains in the possession of the corresponding author&#46;</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&#46;</p></span></span>"
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Idiomas
Reumatología Clínica (English Edition)
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