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Curi Tuma, S. González Bombardiere, S. Silva-Valenzuela" "autores" => array:4 [ 0 => array:4 [ "nombre" => "C." "apellidos" => "del Puerto Troncoso" "email" => array:2 [ 0 => "mcdelpue@uc.cl" 1 => "conildelpuerto@gmail.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" => "Curi Tuma" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "S." "apellidos" => "González Bombardiere" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] 3 => array:3 [ "nombre" => "S." "apellidos" => "Silva-Valenzuela" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Department of Dermatology, Faculty of Medicine, Pontificia Universidad Católica de Chile, Chile" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Chilean Safety Association [<span class="elsevierStyleItalic">Asociación Chilena de Seguridad</span>], Santiago, Chile" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Department of Pathology, Faculty of Medicine, Pontificia Universidad Católica de Chile, Chile" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Eritema figurado neutrofílico de la infancia asociado a Leucemia Mielomonocítica Juvenil" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 468 "Ancho" => 1301 "Tamanyo" => 293633 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Histological examination of a biopsy specimen taken from an indurated border of one plaque, hematoxylin–eosin stain (a) superficial and deep perivascular and interstitial infiltrate, without epidermal changes (Hematoxilina-Eosina 80X). (b) The deep dermal infiltrate was composed predominantly of neutrophils, with abundant nuclear dust, without other signs of vasculitis (Hematoxilina-Eosina 100X).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">A 2-year-old girl presented with a 3-month history of asymptomatic annular erythematous skin eruptions. Each plaque began as a small erythematous papule that enlarged, acquiring an annular configuration with central clearing. During this time some lesions spontaneously disappeared, leaving hyperpigmentation. The patient did not present constitutional symptoms. At the age of 1, she had been diagnosed with Disseminated Juvenile Xantogranuloma, without neurologic, ophthalmologic or other visceral involvement. She was otherwise healthy, had neither allergies nor a significant family history. There was no history of insect bites or exposure to animals and she had not received any medications previous to the eruption.</p><p id="par0010" class="elsevierStylePara elsevierViewall">Physical examination revealed multiple annular and polycyclic erythematous plaques, with indurated borders and petechiae on the rims. The plaques were devoid of vesicles, crusts, erosions or desquamation (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>) and were distributed on her upper chest, abdomen and upper back. There was no hepatosplenomegaly, lymphadenopathy, or arthritis.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Laboratory studies disclosed a normal white cell count with normal differential. Immunoglobulin G titers were slightly elevated; IgM, IgA, IgE, antistreptolysin O titers; serum levels of C3 and C4 were normal. Anti-SS-A (Ro) and anti-SS-B (La) antibodies were negative. A stool examination for parasites and serologic tests for Toxocara, Bartonella hensenlae, Epstein–Barr virus and hepatitis B virus infection were negative.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Histological examination of an annular plaque revealed superficial and deep perivascular and interstitial mixed-cell dermatitis. The deep dermal infiltrate was composed predominantly of neutrophils with abundant nuclear dust. Other vasculitis signs were absent (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). Clinical and histopathological features were consistent with Neutrophilic Figurate Erythema of Infancy (NFEI).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">Two months after the consultation, the patient presented clinical and laboratory findings suggestive of mononucleosis syndrome due to Epstein–Barr virus. During the infection, the lesions increased in size and number. No improvement was seen during febrile periods.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Ten months after the beginning of plaques, a complete blood count was performed, which showed leukocytosis: 28,600<span class="elsevierStyleHsp" style=""></span>WBC/mm<span class="elsevierStyleSup">3</span> with 22% monocytes and blast cells. A myelogram showed hypercellularity and 12% blast cells with monocyte-like appearance. The immunophenotype showed 17% immature monocytoid cells. Her fetal hemoglobin concentration was 24%. The translocation study (t9:22, t8:1 and t15:17) was negative. Therefore, juvenile myelomonocytic leukemia (JMML) was diagnosed. She began oral chemotherapy with hydroxiurea, observing an important improvement in the cutaneous lesions.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Discussion</span><p id="par0035" class="elsevierStylePara elsevierViewall">Annular or figurate erythemas of infancy (AEI) are characterized by a primary annular, circinate, arcuate or polycyclic pattern of cutaneous lesions.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> The lesions may be due to a known cause (rheumatic marginated erythema, neonatal lupus, erythema chronicum migrans) or may be idiopathic<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> and may present with a localized or broad distribution.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,3</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">NFEI belongs to those figurate erythemas of unknown etiology.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> It is characterized by papular erythematous eruptions with rapid centrifugal enlargement to annular or polycyclic asymptomatic plaques with indurated borders devoid of vesicles, crusts or desquamation.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,5</span></a> Frequently, the eruptions begin on the face and then spread centrifugally to the limbs. The patches tend to disappear within 2–4 weeks, but the disease course is chronic.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,3</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Histologically, NFEI is characterized by a superficial and deep, perivascular and interstitial infiltrate of neutrophils associated with leukocytoclasis but without other signs of vasculitis.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2,4,5</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Differential diagnosis of NFEI includes annular erythemas of infancy and dermatoses with a prominent neutrophilic infiltrate such as Sweet syndrome (SS) and pyoderma gangrenosum, urticarial lesions of dermatitis herpetiformis or linear IgA dermatosis, early lesions of bullous lupus erythematosus, Still disease, Sjögren's syndrome and early leukocytoclastic vasculitis.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Sweet syndrome (SS) was very important in our differential diagnosis, considering the association with JMML presented in this case. However, our patient lacked typical manifestations of SS, as the lesions were asymptomatic and neither fever nor neutrophilia were present.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Furthermore, paraneoplastic SS in children usually presents mucosal involvement, anemia and thrombocytopenia, and the neoplasm is concomitant with skin eruptions.<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">7,8</span></a> Finally, SS histological findings differ from NFEI, as SS is characterized by a dense nodular or diffuse dermal infiltrate of neutrophils with nuclear dust with a variable amount of lymphocytes, histocytes, extravasated erythrocytes and a Grenz zone,<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,7,8</span></a> findings not found in NFEI descriptions or in this patient.</p><p id="par0060" class="elsevierStylePara elsevierViewall">Some authors have described cases of recurrent annular neutrophilic dermatosis in adulthood<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> and have included this condition in the spectrum of neutrophilic dermatoses.</p><p id="par0065" class="elsevierStylePara elsevierViewall">Although NFEI has been considered a benign chronic condition, only three pediatric cases have been reported.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,4,6</span></a> The association with a hematological neoplasm and its response to its treatment in our patient suggest that NFEI may be among the group of neutrophilic dermatoses. Cytokines and other biochemical mediators produced by blast cells could be involved in the development or maintenance of the skin lesions.</p><p id="par0070" class="elsevierStylePara elsevierViewall">A standard treatment for NFEI has not been established. Previous reported treatments include topical steroids, systemic corticosteroids, hydroxychloroquine and colchicine, with poor responses.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,5</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">We present the first case of NFEI associated with JMML. Histological findings and the association with a hematological neoplasm suggest that NFEI may be in the spectrum of neutrophilic dermatoses. Therefore, after a diagnosis of NFEI an exhaustive study to exclude inflammatory diseases and neoplasms is warranted.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Discussion" ] 1 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:2 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 996 "Ancho" => 802 "Tamanyo" => 93987 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Multiple annular and polycyclic erythematous plaques with purpuric macules and indurated borders devoid of crusts, vesicles or desquamation distributed along her left chest and axillae.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 468 "Ancho" => 1301 "Tamanyo" => 293633 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Histological examination of a biopsy specimen taken from an indurated border of one plaque, hematoxylin–eosin stain (a) superficial and deep perivascular and interstitial infiltrate, without epidermal changes (Hematoxilina-Eosina 80X). (b) The deep dermal infiltrate was composed predominantly of neutrophils, with abundant nuclear dust, without other signs of vasculitis (Hematoxilina-Eosina 100X).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:9 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Approaches to the dermatopathologic diagnosis of figurate lesions" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "J.J. Ríos-Martín" 1 => "L. Ferrándiz-Pulido" 2 => "D. 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Year/Month | Html | Total | |
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2024 November | 5 | 6 | 11 |
2024 October | 64 | 40 | 104 |
2024 September | 87 | 20 | 107 |
2024 August | 106 | 57 | 163 |
2024 July | 118 | 36 | 154 |
2024 June | 110 | 38 | 148 |
2024 May | 95 | 32 | 127 |
2024 April | 86 | 26 | 112 |
2024 March | 100 | 36 | 136 |
2024 February | 80 | 30 | 110 |
2024 January | 58 | 30 | 88 |
2023 December | 85 | 13 | 98 |
2023 November | 76 | 25 | 101 |
2023 October | 88 | 21 | 109 |
2023 September | 99 | 31 | 130 |
2023 August | 49 | 12 | 61 |
2023 July | 96 | 34 | 130 |
2023 June | 75 | 21 | 96 |
2023 May | 73 | 23 | 96 |
2023 April | 53 | 15 | 68 |
2023 March | 45 | 37 | 82 |
2023 February | 47 | 27 | 74 |
2023 January | 44 | 30 | 74 |
2022 December | 56 | 42 | 98 |
2022 November | 58 | 29 | 87 |
2022 October | 39 | 25 | 64 |
2022 September | 74 | 34 | 108 |
2022 August | 27 | 42 | 69 |
2022 July | 42 | 37 | 79 |
2022 June | 52 | 24 | 76 |
2022 May | 150 | 24 | 174 |
2022 April | 147 | 52 | 199 |
2022 March | 110 | 46 | 156 |
2022 February | 77 | 25 | 102 |
2022 January | 115 | 39 | 154 |
2021 December | 88 | 36 | 124 |
2021 November | 84 | 52 | 136 |
2021 October | 108 | 45 | 153 |
2021 September | 89 | 38 | 127 |
2021 August | 79 | 29 | 108 |
2021 July | 75 | 27 | 102 |
2021 June | 63 | 22 | 85 |
2021 May | 49 | 35 | 84 |
2021 April | 130 | 60 | 190 |
2021 March | 105 | 35 | 140 |
2021 February | 65 | 24 | 89 |
2021 January | 57 | 22 | 79 |
2020 December | 50 | 19 | 69 |
2020 November | 29 | 25 | 54 |
2020 October | 32 | 18 | 50 |
2020 September | 30 | 17 | 47 |
2020 August | 28 | 22 | 50 |
2020 July | 32 | 12 | 44 |
2020 June | 33 | 27 | 60 |
2020 May | 26 | 25 | 51 |
2020 April | 26 | 18 | 44 |
2020 March | 25 | 26 | 51 |
2020 February | 5 | 3 | 8 |
2020 January | 3 | 0 | 3 |
2019 December | 4 | 4 | 8 |
2019 November | 4 | 1 | 5 |
2019 September | 3 | 2 | 5 |
2019 August | 4 | 1 | 5 |
2019 July | 4 | 3 | 7 |
2019 June | 4 | 6 | 10 |
2019 May | 5 | 6 | 11 |
2019 April | 2 | 10 | 12 |
2019 March | 2 | 7 | 9 |
2019 February | 1 | 2 | 3 |
2019 January | 4 | 0 | 4 |
2018 December | 0 | 5 | 5 |
2018 November | 1 | 0 | 1 |
2018 October | 4 | 0 | 4 |
2018 September | 5 | 0 | 5 |
2018 March | 4 | 0 | 4 |
2018 February | 44 | 4 | 48 |
2018 January | 44 | 3 | 47 |
2017 December | 36 | 7 | 43 |
2017 November | 43 | 2 | 45 |
2017 October | 48 | 6 | 54 |
2017 September | 22 | 3 | 25 |
2017 August | 43 | 9 | 52 |
2017 July | 35 | 13 | 48 |
2017 June | 30 | 6 | 36 |
2017 May | 37 | 12 | 49 |
2017 April | 28 | 9 | 37 |
2017 March | 30 | 6 | 36 |
2017 February | 53 | 7 | 60 |
2017 January | 24 | 6 | 30 |
2016 December | 25 | 5 | 30 |
2016 November | 27 | 8 | 35 |
2016 October | 21 | 7 | 28 |
2016 September | 0 | 11 | 11 |
2016 August | 0 | 3 | 3 |
2016 July | 5 | 2 | 7 |
2016 June | 16 | 2 | 18 |
2016 May | 10 | 3 | 13 |
2016 April | 14 | 22 | 36 |
2016 March | 5 | 1 | 6 |
2016 February | 15 | 1 | 16 |
2016 January | 12 | 0 | 12 |
2015 December | 8 | 2 | 10 |
2015 November | 0 | 2 | 2 |
2015 October | 0 | 3 | 3 |
2015 September | 0 | 2 | 2 |
2015 August | 0 | 2 | 2 |
2015 July | 1 | 1 | 2 |
2015 June | 6 | 6 | 12 |