Case for Diagnosis
Máculas evanescentes en tronco y extremidades
Fleeting Macules on the Trunk and Limbs
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Flores-Terry, M. Franco-Muñóz, J.A. Garrido-Martín, N. Villasanti-Rivas" "autores" => array:4 [ 0 => array:4 [ "nombre" => "M.Á." "apellidos" => "Flores-Terry" "email" => array:1 [ 0 => "miguelterry85@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" ] ] ] 1 => array:3 [ "nombre" => "M." "apellidos" => "Franco-Muñóz" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "J.A." "apellidos" => "Garrido-Martín" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 3 => array:3 [ "nombre" => "N." "apellidos" => "Villasanti-Rivas" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital General Universitario de Ciudad Real, Ciudad Real, España" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Hospital General Universitario de Ciudad Real, Ciudad Real, España" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Fleeting Macules on the Trunk and Limbs" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:6 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 300 "Ancho" => 400 "Tamanyo" => 20545 ] ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Medical History</span><p id="par0005" class="elsevierStylePara elsevierViewall">A 44-year-old man was assessed for a skin rash associated with evening fever and joint pain that had appeared 7 days earlier. He reported catarrhal symptoms and pharyngeal pain in the previous week. He worked as a livestock farmer and stated that he had not traveled recently or any risky sexual behavior. Physical examination showed a salmon-colored maculopapular rash mainly affecting the trunk (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>) and the proximal areas of the upper and lower limbs (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>), with sparing of the palms and soles. The patient also had fever (><span class="elsevierStyleHsp" style=""></span>39°<span class="elsevierStyleSmallCaps">C</span>) and arthritis affecting the joints of his right shoulder and left knee and ankle. No enlargement was noticed in the axillary or inguinal lymph nodes or in the liver or spleen. Skin biopsy showed a normal epidermis with a superficial perivascular lymphohistiocytic dermal infiltrate with neutrophils. No other abnormal findings were observed (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Additional Tests</span><p id="par0010" class="elsevierStylePara elsevierViewall">Complete blood count with leukocytes, 13<span class="elsevierStyleHsp" style=""></span>300<span class="elsevierStyleHsp" style=""></span>leukocytes/μL (segment, 83.7%); erythrocyte sedimentation rate, 15<span class="elsevierStyleHsp" style=""></span>mm; biochemistry with aspartate aminotransferase, 22<span class="elsevierStyleHsp" style=""></span>IU/L; alanine aminotransferase, 20<span class="elsevierStyleHsp" style=""></span>IU/L; ferritin, 1148<span class="elsevierStyleHsp" style=""></span>ng/mL; and C-reactive protein, 13.7<span class="elsevierStyleHsp" style=""></span>mg/dL. Antinuclear antibodies, rheumatoid factor, microbial serology, and blood and urine cultures were all negative. Complement, immunoglobulin, and protein levels were normal.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">What Is Your diagnosis?</span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Diagnosis</span><p id="par0015" class="elsevierStylePara elsevierViewall">Adult-onset Still disease.</p></span></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Clinical Course and Treatment</span><p id="par0020" class="elsevierStylePara elsevierViewall">The findings were compatible with a diagnosis of adult-onset Still disease (AOSD).<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> The patient was started on naproxen 500<span class="elsevierStyleHsp" style=""></span>mg/12<span class="elsevierStyleHsp" style=""></span>h and a tapering course of prednisone 20<span class="elsevierStyleHsp" style=""></span>mg/d. He showed initial improvement, but the disease flared up again when the corticosteroid doses were reduced. Methotrexate 10<span class="elsevierStyleHsp" style=""></span>mg/wk was added to the treatment. At the time of writing, after a year of follow-up, the patient has achieved good clinical control of the disease with prednisone 5<span class="elsevierStyleHsp" style=""></span>mg/d and methotrexate 2.5<span class="elsevierStyleHsp" style=""></span>mg/wk.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Comment</span><p id="par0025" class="elsevierStylePara elsevierViewall">AOSD is an uncommon disease described by Bywaters in 1971.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> It was recently classified as a polygenic autoinflammatory disease.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> It generally appears between the third and sixth decade of life and is more common in women. Its pathophysiology and triggers remain unclear,<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> although several theories have been proposed,<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> including infections, which would largely act as viral “activators”. A number of genetic markers (HLA DRB1*1201, 1501, B35, DR2, DR5) have also been implicated.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> Clinically, the disease is characterized by a fever that spikes in the evening, an evanescent salmon-colored maculopapular rash, arthritis, enlarged lymph nodes, and/or hepatosplenomegaly. The differential diagnosis should include numerous entities such as urticaria, vasculitis, drug eruptions, Sweet syndrome, and hematologic tumors. The hallmark histopathologic finding is a perivascular lymphohistiocytic dermal infiltrate with neutrophils. Remarkable findings in the blood tests are leukocytosis, hyperferritinemia, and elevated liver enzymes. Antinuclear antibodies and rheumatoid factor are negative.<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">3,4</span></a> There may also be high levels of interleukin (IL) 6, tumor necrosis factor, interferon-γ, and IL-8.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> Although ferritin levels have low specificity and specificity, they generally exceed 1000<span class="elsevierStyleHsp" style=""></span>ng/mL and correlate with disease activity. They typically return to normal on remission of the disease<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> and they may even predict a chronic course.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a> Glycosylated ferritin is a more specific marker. Levels of the kinase protein MAP4K3 might be correlated with disease activity and may even have a pathogenic role<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">AOSD can be divided into 2 main phenotypes, systemic-onset AOSD and articular AOSD, based on disease course and cytokine profile.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> Complications are rare<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> but can include hepatitis, pleuritis, pneumonitis, and carditis. More serious complications are macrophage activation syndrome and disseminated intravascular coagulation, both of which have a high mortality. In 1 series, 14.8% of patients developed severe complications<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> and female sex was the greatest risk factor.<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1,3</span></a> Pancytopenia and respiratory distress syndrome should raise suspicion of the possibility of macrophage activation syndrome.</p><p id="par0035" class="elsevierStylePara elsevierViewall">The treatment for OASD is based on experiences with individual patients and/or small series.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> Pharmacologic treatments include nonsteroidal anti-inflammatory drugs, corticosteroids, methotrexate, hydroxychloroquine, leflunomide, azathioprine, ciclosporin, penicillamine, and tacrolimus. Biologic drugs have also been used in severe, refractory cases and include infliximab, etanercept, adalimumab, anakinra, and tocilizumab.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Conflicts of Interest</span><p id="par0040" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:7 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Medical History" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Additional Tests" ] 2 => array:3 [ "identificador" => "sec0015" "titulo" => "What Is Your diagnosis?" "secciones" => array:1 [ 0 => array:2 [ "identificador" => "sec0020" "titulo" => "Diagnosis" ] ] ] 3 => array:2 [ "identificador" => "sec0025" "titulo" => "Clinical Course and Treatment" ] 4 => array:2 [ "identificador" => "sec0030" "titulo" => "Comment" ] 5 => array:2 [ "identificador" => "sec0035" "titulo" => "Conflicts of Interest" ] 6 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Flores-Terry MÁ, Franco-Muñóz M, Garrido-Martín JA, Villasanti-Rivas N. Máculas evanescentes en tronco y extremidades. Actas Dermosifiliogr. 2018;109:645–646.</p>" ] ] "multimedia" => array:3 [ 0 => array:6 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 300 "Ancho" => 400 "Tamanyo" => 18594 ] ] ] 1 => array:6 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 300 "Ancho" => 400 "Tamanyo" => 20545 ] ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 400 "Ancho" => 1152 "Tamanyo" => 165858 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, Hematoxylin-eosin, original magnification ×10; B, Hematoxylin-eosin, original magnification ×40.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:6 [ 0 => array:3 [ "identificador" => "bib0035" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Epidemiological study of adult-onset Still's disease using a Japanese administrative database" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "N. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 8 | 7 | 15 |
| 2024 October | 65 | 30 | 95 |
| 2024 September | 69 | 17 | 86 |
| 2024 August | 73 | 47 | 120 |
| 2024 July | 68 | 29 | 97 |
| 2024 June | 66 | 30 | 96 |
| 2024 May | 52 | 32 | 84 |
| 2024 April | 78 | 27 | 105 |
| 2024 March | 72 | 24 | 96 |
| 2024 February | 56 | 34 | 90 |
| 2024 January | 57 | 25 | 82 |
| 2023 December | 59 | 17 | 76 |
| 2023 November | 92 | 23 | 115 |
| 2023 October | 59 | 26 | 85 |
| 2023 September | 69 | 34 | 103 |
| 2023 August | 37 | 19 | 56 |
| 2023 July | 70 | 36 | 106 |
| 2023 June | 54 | 20 | 74 |
| 2023 May | 68 | 33 | 101 |
| 2023 April | 35 | 30 | 65 |
| 2023 March | 54 | 22 | 76 |
| 2023 February | 41 | 26 | 67 |
| 2023 January | 42 | 43 | 85 |
| 2022 December | 50 | 38 | 88 |
| 2022 November | 28 | 29 | 57 |
| 2022 October | 33 | 20 | 53 |
| 2022 September | 21 | 35 | 56 |
| 2022 August | 38 | 35 | 73 |
| 2022 July | 26 | 39 | 65 |
| 2022 June | 36 | 31 | 67 |
| 2022 May | 35 | 40 | 75 |
| 2022 April | 35 | 38 | 73 |
| 2022 March | 35 | 47 | 82 |
| 2022 February | 29 | 26 | 55 |
| 2022 January | 27 | 43 | 70 |
| 2021 December | 37 | 48 | 85 |
| 2021 November | 41 | 54 | 95 |
| 2021 October | 29 | 43 | 72 |
| 2021 September | 28 | 44 | 72 |
| 2021 August | 31 | 43 | 74 |
| 2021 July | 31 | 30 | 61 |
| 2021 June | 30 | 41 | 71 |
| 2021 May | 38 | 54 | 92 |
| 2021 April | 96 | 79 | 175 |
| 2021 March | 59 | 45 | 104 |
| 2021 February | 70 | 37 | 107 |
| 2021 January | 36 | 38 | 74 |
| 2020 December | 32 | 38 | 70 |
| 2020 November | 30 | 36 | 66 |
| 2020 October | 26 | 14 | 40 |
| 2020 September | 20 | 26 | 46 |
| 2020 August | 31 | 29 | 60 |
| 2020 July | 27 | 20 | 47 |
| 2020 June | 30 | 40 | 70 |
| 2020 May | 24 | 22 | 46 |
| 2020 April | 14 | 11 | 25 |
| 2020 March | 22 | 18 | 40 |
| 2020 February | 1 | 0 | 1 |
| 2018 October | 1 | 0 | 1 |
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