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Padilla-España, J. del Boz-González, M. de Troya Martín" "autores" => array:3 [ 0 => array:4 [ "nombre" => "L." "apellidos" => "Padilla-España" "email" => array:1 [ 0 => "laupadesp@gmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "J." "apellidos" => "del Boz-González" ] 2 => array:2 [ "nombre" => "M." "apellidos" => "de Troya Martín" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Dermatología, Hospital Costa del Sol, Marbella, Málaga, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Pápulas mucosas en una niña de 9 años" ] ] "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" => 1230 "Ancho" => 1651 "Tamanyo" => 646960 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A, Hematoxylin-eosin, original magnification ×100. B, Hematoxylin-eosin, original magnification ×200. C, Neurofilament immunohistochemistry. D, S100 immunohistochemistry.</p>" ] ] ] "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 9-year-old girl with no family history of interest was undergoing follow-up in pediatrics for functional chronic constipation since childhood. She was referred to dermatology for multiple lesions on the tip of the tongue that had developed at 7 years of age.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">The initial physical examination revealed marfanoid habitus (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A), with thickening and mild eversion of the lip and the free margin of the eyelid. Several firm, opaque, papular lesions were observed on the tip of the tongue (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>B). A single skin-colored papular lesion was observed in the right nasal choana (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a><span class="elsevierStyleHsp" style=""></span>C) and several others on the lips (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Additional Tests</span><p id="par0015" class="elsevierStylePara elsevierViewall">A punch biopsy of one of the lingual papules revealed well-circumscribed nodular lesions in the dermis, consisting of hyperplastic nerve fibers that were positive for neurofilament and S100 staining (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Based on these histological finding magnetic resonance imaging of the adrenal glands was performed, and levels of urine catecholamines and blood calcitonin were determined. All test results were normal. However, thyroid ultrasound revealed 3 vascularized, hypodense, bilateral nodules, strongly suggesting malignancy. A genetic study revealed a mutation in the <span class="elsevierStyleItalic">RET</span> proto-oncogene (p.Met918Thr).</p><p id="par0025" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">What Is Your Diagnosis?</span></p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Mucosal neuromas in a case of multiple endocrine neoplasia.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Course</span><p id="par0035" class="elsevierStylePara elsevierViewall">Based on the histological confirmation of mucosal neuromas and the patient́s clinical history of chronic constipation, several additional tests were conducted to confirm the presence of multiple endocrine neoplasia (MEN). A thyroidectomy, performed given the strong suspicion of malignant infiltration of the thyroid, confirmed the presence of medullary thyroid cancer. The final diagnosis was MEN type 2B with cutaneous, thyroid, and gastrointestinal involvement.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Comments</span><p id="par0040" class="elsevierStylePara elsevierViewall">MEN type 2B syndrome is an inherited autosomal dominant disorder caused by a mutation in the <span class="elsevierStyleItalic">RET</span> proto-oncogene.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">1</span></a> MEN type 2 is classified into 3 types: 2A, 2B, and familial medullary thyroid cancer. MEN type 2B has a low incidence (1 case per 200 000), and represents 5% of all MEN type 2 cases. This disorder is characterized by endocrine neoplasias such as pheochromocytoma (50% of cases) and medullary thyroid cancer (95% of cases).<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">2</span></a> The latter is usually the first neoplasia to develop, and has a poor prognosis owing to its aggressive and multicentric nature and its high rate of metastasis (mainly in the lung and liver). A prophylactic thyroidectomy is thus recommended in such cases.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">2</span></a> MEN type 2B is also characterized by intestinal ganglioneuromatosis resulting in chronic constipation, and a peculiar phenotype with marfanoid habitus and thickening of the lips and eyelids, as seen in our patient.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">3</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Mucosal neuromas are the guiding sign for the early diagnosis of this disease, and are usually located in the midface region, predominantly in the lingual and labial mucosa, and less frequently in the conjunctiva and palate. In severe cases, mucosal neuromas may be present at birth; the number and/or size of these neuromas may increase over time or remain unchanged.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">4</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Mucosal neuromas, given their almost pathognomonic character, can thus constitute the key to early identification of MEN. The dermatologist plays a crucial role in such cases.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Conflicts of Interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:8 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Medical History" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Physical Examination" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Additional Tests" ] 3 => array:2 [ "identificador" => "sec0020" "titulo" => "Diagnosis" ] 4 => array:2 [ "identificador" => "sec0025" "titulo" => "Course" ] 5 => array:2 [ "identificador" => "sec0030" "titulo" => "Comments" ] 6 => array:2 [ "identificador" => "sec0035" "titulo" => "Conflicts of Interest" ] 7 => 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: Padilla-España L, del Boz-González J, de Troya Martín M. Pápulas mucosas en una niña de 9 años. Actas Dermosifiliogr. 2015;106:227–228.</p>" ] ] "multimedia" => array:2 [ 0 => array:6 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 665 "Ancho" => 991 "Tamanyo" => 15091 ] ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1230 "Ancho" => 1651 "Tamanyo" => 646960 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A, Hematoxylin-eosin, original magnification ×100. B, Hematoxylin-eosin, original magnification ×200. C, Neurofilament immunohistochemistry. D, S100 immunohistochemistry.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:4 [ 0 => array:3 [ "identificador" => "bib0025" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Multiple endocrine neoplasia type 2: An overview" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "J. Moline" 1 => "C. 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Kim" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.5021/ad.2010.22.4.452" "Revista" => array:6 [ "tituloSerie" => "Ann Dermatol" "fecha" => "2010" "volumen" => "22" "paginaInicial" => "452" "paginaFinal" => "455" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21165219" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0035" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Update multiple endocrine neoplasia type 2" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "F. Raue" 1 => "K. Frank-Raue" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1007/s10689-010-9320-2" "Revista" => array:6 [ "tituloSerie" => "Fam Cancer" "fecha" => "2010" "volumen" => "9" "paginaInicial" => "449" "paginaFinal" => "457" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/20087666" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0040" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "A family presenting with multiple endocrine neoplasia type 2<span class="elsevierStyleHsp" style=""></span>B: A case report" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "M. Majidi" 1 => "V. Haghpanah" 2 => "M. Hedayati" 3 => "P. Khashayar" 4 => "M.R. Mohajeri-Tehrani" 5 => "B. Larijani" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1186/1752-1947-5-587" "Revista" => array:5 [ "tituloSerie" => "J Med Case Rep" "fecha" => "2011" "volumen" => "5" "paginaInicial" => "587" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22185228" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/15782190/0000010600000003/v3_202209240641/S1578219015000219/v3_202209240641/en/main.assets" "Apartado" => array:4 [ "identificador" => "6171" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Cases for Diagnosis" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/15782190/0000010600000003/v3_202209240641/S1578219015000219/v3_202209240641/en/main.pdf?idApp=UINPBA000044&text.app=https://actasdermo.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1578219015000219?idApp=UINPBA000044" ]
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2024 March | 69 | 22 | 91 |
2024 February | 46 | 31 | 77 |
2024 January | 48 | 31 | 79 |
2023 December | 56 | 13 | 69 |
2023 November | 51 | 26 | 77 |
2023 October | 51 | 20 | 71 |
2023 September | 54 | 33 | 87 |
2023 August | 34 | 20 | 54 |
2023 July | 40 | 34 | 74 |
2023 June | 47 | 24 | 71 |
2023 May | 46 | 19 | 65 |
2023 April | 44 | 16 | 60 |
2023 March | 38 | 26 | 64 |
2023 February | 36 | 21 | 57 |
2023 January | 41 | 25 | 66 |
2022 December | 48 | 28 | 76 |
2022 November | 22 | 21 | 43 |
2022 October | 25 | 20 | 45 |
2022 September | 30 | 41 | 71 |
2022 August | 43 | 36 | 79 |
2022 July | 21 | 31 | 52 |
2022 June | 39 | 33 | 72 |
2022 May | 32 | 31 | 63 |
2022 April | 44 | 31 | 75 |
2022 March | 44 | 45 | 89 |
2022 February | 33 | 20 | 53 |
2022 January | 38 | 39 | 77 |
2021 December | 27 | 34 | 61 |
2021 November | 40 | 34 | 74 |
2021 October | 45 | 47 | 92 |
2021 September | 33 | 31 | 64 |
2021 August | 51 | 25 | 76 |
2021 July | 39 | 21 | 60 |
2021 June | 28 | 26 | 54 |
2021 May | 32 | 24 | 56 |
2021 April | 43 | 52 | 95 |
2021 March | 59 | 12 | 71 |
2021 February | 58 | 20 | 78 |
2021 January | 28 | 7 | 35 |
2020 December | 27 | 12 | 39 |
2020 November | 24 | 6 | 30 |
2020 October | 18 | 10 | 28 |
2020 September | 11 | 9 | 20 |
2020 August | 26 | 11 | 37 |
2020 July | 18 | 12 | 30 |
2020 June | 33 | 14 | 47 |
2020 May | 25 | 16 | 41 |
2020 April | 25 | 13 | 38 |
2020 March | 23 | 12 | 35 |
2020 February | 2 | 0 | 2 |
2020 January | 4 | 1 | 5 |
2019 December | 8 | 2 | 10 |
2019 November | 4 | 0 | 4 |
2019 October | 0 | 1 | 1 |
2019 September | 4 | 1 | 5 |
2019 August | 4 | 0 | 4 |
2019 July | 4 | 0 | 4 |
2019 June | 5 | 1 | 6 |
2019 May | 4 | 13 | 17 |
2019 April | 2 | 1 | 3 |
2019 March | 4 | 5 | 9 |
2019 February | 3 | 0 | 3 |
2019 January | 4 | 0 | 4 |
2018 November | 2 | 0 | 2 |
2018 October | 6 | 0 | 6 |
2018 September | 1 | 0 | 1 |
2018 February | 16 | 1 | 17 |
2018 January | 29 | 5 | 34 |
2017 December | 30 | 7 | 37 |
2017 November | 18 | 4 | 22 |
2017 October | 25 | 2 | 27 |
2017 September | 19 | 3 | 22 |
2017 August | 19 | 4 | 23 |
2017 July | 20 | 2 | 22 |
2017 June | 29 | 8 | 37 |
2017 May | 26 | 8 | 34 |
2017 April | 25 | 13 | 38 |
2017 March | 13 | 6 | 19 |
2017 February | 15 | 6 | 21 |
2017 January | 15 | 5 | 20 |
2016 December | 29 | 11 | 40 |
2016 November | 48 | 10 | 58 |
2016 October | 26 | 5 | 31 |
2016 September | 0 | 3 | 3 |
2016 July | 13 | 1 | 14 |
2016 June | 12 | 0 | 12 |
2016 May | 13 | 4 | 17 |
2016 April | 7 | 1 | 8 |
2016 March | 3 | 0 | 3 |
2016 February | 11 | 1 | 12 |
2016 January | 12 | 1 | 13 |
2015 December | 15 | 0 | 15 |
2015 November | 6 | 0 | 6 |
2015 October | 4 | 1 | 5 |
2015 August | 0 | 2 | 2 |
2015 July | 7 | 4 | 11 |
2015 June | 6 | 2 | 8 |
2015 May | 4 | 5 | 9 |
2015 April | 2 | 20 | 22 |