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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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año/Mes | Html | Total | |
---|---|---|---|
2024 Noviembre | 4 | 6 | 10 |
2024 Octubre | 64 | 40 | 104 |
2024 Septiembre | 87 | 20 | 107 |
2024 Agosto | 106 | 57 | 163 |
2024 Julio | 118 | 36 | 154 |
2024 Junio | 110 | 38 | 148 |
2024 Mayo | 95 | 32 | 127 |
2024 Abril | 86 | 26 | 112 |
2024 Marzo | 100 | 36 | 136 |
2024 Febrero | 80 | 30 | 110 |
2024 Enero | 58 | 30 | 88 |
2023 Diciembre | 85 | 13 | 98 |
2023 Noviembre | 76 | 25 | 101 |
2023 Octubre | 88 | 21 | 109 |
2023 Septiembre | 99 | 31 | 130 |
2023 Agosto | 49 | 12 | 61 |
2023 Julio | 96 | 34 | 130 |
2023 Junio | 75 | 21 | 96 |
2023 Mayo | 73 | 23 | 96 |
2023 Abril | 53 | 15 | 68 |
2023 Marzo | 45 | 37 | 82 |
2023 Febrero | 47 | 27 | 74 |
2023 Enero | 44 | 30 | 74 |
2022 Diciembre | 56 | 42 | 98 |
2022 Noviembre | 58 | 29 | 87 |
2022 Octubre | 39 | 25 | 64 |
2022 Septiembre | 74 | 34 | 108 |
2022 Agosto | 27 | 42 | 69 |
2022 Julio | 42 | 37 | 79 |
2022 Junio | 52 | 24 | 76 |
2022 Mayo | 150 | 24 | 174 |
2022 Abril | 147 | 52 | 199 |
2022 Marzo | 110 | 46 | 156 |
2022 Febrero | 77 | 25 | 102 |
2022 Enero | 115 | 39 | 154 |
2021 Diciembre | 88 | 36 | 124 |
2021 Noviembre | 84 | 52 | 136 |
2021 Octubre | 108 | 45 | 153 |
2021 Septiembre | 89 | 38 | 127 |
2021 Agosto | 79 | 29 | 108 |
2021 Julio | 75 | 27 | 102 |
2021 Junio | 63 | 22 | 85 |
2021 Mayo | 49 | 35 | 84 |
2021 Abril | 130 | 60 | 190 |
2021 Marzo | 105 | 35 | 140 |
2021 Febrero | 65 | 24 | 89 |
2021 Enero | 57 | 22 | 79 |
2020 Diciembre | 50 | 19 | 69 |
2020 Noviembre | 29 | 25 | 54 |
2020 Octubre | 32 | 18 | 50 |
2020 Septiembre | 30 | 17 | 47 |
2020 Agosto | 28 | 22 | 50 |
2020 Julio | 32 | 12 | 44 |
2020 Junio | 33 | 27 | 60 |
2020 Mayo | 26 | 25 | 51 |
2020 Abril | 26 | 18 | 44 |
2020 Marzo | 25 | 26 | 51 |
2020 Febrero | 5 | 3 | 8 |
2020 Enero | 3 | 0 | 3 |
2019 Diciembre | 4 | 4 | 8 |
2019 Noviembre | 4 | 1 | 5 |
2019 Septiembre | 3 | 2 | 5 |
2019 Agosto | 4 | 1 | 5 |
2019 Julio | 4 | 3 | 7 |
2019 Junio | 4 | 6 | 10 |
2019 Mayo | 5 | 6 | 11 |
2019 Abril | 2 | 10 | 12 |
2019 Marzo | 2 | 7 | 9 |
2019 Febrero | 1 | 2 | 3 |
2019 Enero | 4 | 0 | 4 |
2018 Diciembre | 0 | 5 | 5 |
2018 Noviembre | 1 | 0 | 1 |
2018 Octubre | 4 | 0 | 4 |
2018 Septiembre | 5 | 0 | 5 |
2018 Marzo | 4 | 0 | 4 |
2018 Febrero | 44 | 4 | 48 |
2018 Enero | 44 | 3 | 47 |
2017 Diciembre | 36 | 7 | 43 |
2017 Noviembre | 43 | 2 | 45 |
2017 Octubre | 48 | 6 | 54 |
2017 Septiembre | 22 | 3 | 25 |
2017 Agosto | 43 | 9 | 52 |
2017 Julio | 35 | 13 | 48 |
2017 Junio | 30 | 6 | 36 |
2017 Mayo | 37 | 12 | 49 |
2017 Abril | 28 | 9 | 37 |
2017 Marzo | 30 | 6 | 36 |
2017 Febrero | 53 | 7 | 60 |
2017 Enero | 24 | 6 | 30 |
2016 Diciembre | 25 | 5 | 30 |
2016 Noviembre | 27 | 8 | 35 |
2016 Octubre | 21 | 7 | 28 |
2016 Septiembre | 0 | 11 | 11 |
2016 Agosto | 0 | 3 | 3 |
2016 Julio | 5 | 2 | 7 |
2016 Junio | 16 | 2 | 18 |
2016 Mayo | 10 | 3 | 13 |
2016 Abril | 14 | 22 | 36 |
2016 Marzo | 5 | 1 | 6 |
2016 Febrero | 15 | 1 | 16 |
2016 Enero | 12 | 0 | 12 |
2015 Diciembre | 8 | 2 | 10 |
2015 Noviembre | 0 | 2 | 2 |
2015 Octubre | 0 | 3 | 3 |
2015 Septiembre | 0 | 2 | 2 |
2015 Agosto | 0 | 2 | 2 |
2015 Julio | 1 | 1 | 2 |
2015 Junio | 6 | 6 | 12 |