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It is frequently associated with extra cutaneous manifestations, out of which paraproteinemia type IgG λ is the most frequent.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> There is neurological deterioration in 10–15% of cases, without consensus on the most effective type of intervention for patients who present it.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">We present the case of a 59-year-old female patient, with a 5-month history of developing erythematous-plaque-type injuries infiltrated in the face, upper third of the torso and upper extremities, with bilateral oedema of the hands (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>), significant consciousness deterioration, and aphasia and dysarthria 2 days before admission.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">During the neurological assessment, the patient was awake, fully space-oriented but partially time-oriented, with bradypsychia and inattention. She followed simple commands, with preserved nomination and repetition, but significant ideomotor apraxia. The cranial pairs assessment did not show any significant alteration. The motor assessment showed symmetric bilateral postural fine tremor, without any additional alterations. Sensitivity was preserved, with no signs of brain deterioration or meningeal signs.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Her admission tests showed thyrotropin 1.24<span class="elsevierStyleHsp" style=""></span>uUI/ml (normal range 0.3–4.2), vitamin B levels<span class="elsevierStyleInf">12</span> 280<span class="elsevierStyleHsp" style=""></span>pg/ml (normal range 200–900), normal cerebrospinal fluid (glucose 60<span class="elsevierStyleHsp" style=""></span>mg/dl, protein 42<span class="elsevierStyleHsp" style=""></span>mg/dl, leukocytes 3<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleSup">−3</span> negative for oligoclonal bands, adenosine deaminase<3<span class="elsevierStyleHsp" style=""></span>U/I, negative cultures, antistreptolysin O 79.9<span class="elsevierStyleHsp" style=""></span>UI/ml (normal <200), creatinine 0.53<span class="elsevierStyleHsp" style=""></span>mg/dl (normal 0.5–0.9), C-reactive protein 0.22<span class="elsevierStyleHsp" style=""></span>mg/dl (normal <0.5), haemogram and plasma electrolytes within normal ranges, negative rheumatoid factor, C3 and C4 complement components within normal ranges, negative antiphospholipid antibodies and anti-B2-glycoprotein 1. The brain MRI, electroencephalogram, and lumbar puncture did not show significant findings.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Scleromyxedema was suggested as a diagnostic hypothesis. A skin biopsy was performed, showing skin with normal orthokeratosis and epidermis. Dermis with fibrosis, mucin reserves, and band superficial dermis proliferation of fibroblasts. Superficial and deep perivascular mild lymphocitary infiltrate. Preserved annex structures. The immunohistochemical test with monoclonal antibodies showed a positive reaction for CD34 and a negative reaction for actin and CD10, confirming the suspected diagnosis of scleromyxedema. An intravenous methylprednisolone pulse treatment was started (500<span class="elsevierStyleHsp" style=""></span>mg for 2 days). The patient showed progress, with a decrease in skin injuries, but suffered an episode of convulsion, and qualitative consciousness deterioration. The test was completed with electrophoresis and protein immunofixation in blood and urine, showing the presence of the IgG λ monoclonal component, with a myelogram showing bone marrow global hyperplasia. In the face of these clinical findings, and given the progressive deterioration of the patient's consciousness, an intravenous immunoglobulin treatment was initiated at 0.5<span class="elsevierStyleHsp" style=""></span>g/Kg/day dose for 4 days, in addition to the oral prednisone at a 1<span class="elsevierStyleHsp" style=""></span>mg/Kg/day dose. The patient showed progress, with a decrease in skin injuries and consciousness deterioration. No side effects associated with the indicated treatment were detected.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Neurological deterioration related to scleromyxedema includes confusion, dizziness, and dysarthria, ascending paralysis, memory loss, acute psychosis, convulsions and coma.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> The triad of high fever, convulsions and coma, with a prodrome of flu-like symptoms, is known as dermato-neuro syndrome.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> Most cases of scleromyxedema associated with neurological symptoms, such as the case we present here, do not meet the criteria to be considered dermato-neuro syndrome.</p><p id="par0035" class="elsevierStylePara elsevierViewall">There are many treatments for scleromyxedema, including glucocorticoids, retinoid, thalidomide, and extracorporeal photopheresis, phototherapy, cyclosporine, cyclosfamide, melfalan, bortezomid, and stem cell autologous transplantation. The treatments that have shown better results in terms of neurological recovery for patients are plasmapheresis, systemic glucocorticoids and immunoglobulins.<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1–5</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">In conclusion, according to evidence in the medical literature and to the positive results obtained by means of the treatment administered to our patient, we recommend, as a first-line treatment, the use of intravenous immunoglobulins at a dose of 0.5<span class="elsevierStyleHsp" style=""></span>g/kg/day, in addition to the use of systemic glucocorticoids, in patients with neurological deterioration associated with scleromyxedema.</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: Manríquez J, Berroeta-Mauriziano D, Andino-Navarrete R, Vera-Kellet C. Escleromixedema asociado a síntomas neurológicos: tratamiento satisfactorio con inmunoglobulinas. Med Clin (Barc). 2015;144:47–48.</p>" ] ] "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1294 "Ancho" => 2917 "Tamanyo" => 407766 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Erythematous plaques infiltrated in the face, upper-third torso and upper extremities, with bilateral oedema in hands.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0030" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Scleromyxedema: a multicenter study of characteristics, comorbidities, course, and therapy in 30 patients" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "F. 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Hummers" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Medicine (Baltimore)" "fecha" => "2008" "volumen" => "87" "paginaInicial" => "10" "paginaFinal" => "20" ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/23870206/0000014400000001/v1_201509042040/S238702061500011X/v1_201509042040/en/main.assets" "Apartado" => array:4 [ "identificador" => "43309" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Letters to the Editor" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23870206/0000014400000001/v1_201509042040/S238702061500011X/v1_201509042040/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S238702061500011X?idApp=UINPBA00004N" ]
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Vol. 144. Issue 1.
Pages 47-48 (January 2015)
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Vol. 144. Issue 1.
Pages 47-48 (January 2015)
Letter to the Editor
Scleromyxedema with neurological symptoms: Successful treatment with immunoglobulins
Escleromixedema asociado a síntomas neurológicos: tratamiento satisfactorio con inmunoglobulinas
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Juan Manríquez
, Daniela Berroeta-Mauriziano, Romina Andino-Navarrete, Cristián Vera-Kellet
Corresponding author
Departamento de Dermatología, Escuela de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile
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