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(B) Appearance of the donor strip in the parietotemporal region.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "T. Meyer-Gonzalez, C. Bisanga" "autores" => array:2 [ 0 => array:2 [ "nombre" => "T." "apellidos" => "Meyer-Gonzalez" ] 1 => array:2 [ "nombre" => "C." 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Villar, M. Burgués, J.L. Rodríguez-Peralto, R. Rivera, F. Vanaclocha" "autores" => array:5 [ 0 => array:4 [ "nombre" => "M." "apellidos" => "Villar" "email" => array:1 [ 0 => "mariavb20@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" => "Burgués" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "J.L." "apellidos" => "Rodríguez-Peralto" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "R." "apellidos" => "Rivera" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 4 => array:3 [ "nombre" => "F." "apellidos" => "Vanaclocha" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital Universitario 12 de octubre, Madrid, Spain" "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Hospital Universitarion 12 de octubre, Madrid, Spain" "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Amiloidosis nodular primaria cutánea localizada en un paciente con paraproteinemia" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1020 "Ancho" => 1587 "Tamanyo" => 132614 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Well-defined, shiny, reddish-orange plaque with an area of ecchymotic appearance, and 2 firm nodules covered by reddish-orange skin except for one area of ulceration, located in the left lower limb.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">The term primary cutaneous amyloidosis (PCA) refers to a group of diseases caused by the extracellular deposition of amyloid in the skin without the involvement of other organs. PCA has been divided into the following types: macular, lichen and nodular. The first 2 types are characterized by the deposition in the papillary dermis of amyloid derived from the degeneration of keratin filaments. Primary localized cutaneous nodular amyloidosis (PLCNA) is the rarest form of PCA and the only one in which the amyloid deposits are of the amyloid light-chain (AL) type, as in the primary and myeloma-associated systemic forms of amyloidosis. AL amyloidosis is due to monoclonal immunoglobulin (Ig) light-chain deposition.</p><p id="par0010" class="elsevierStylePara elsevierViewall">We report the case of an 83-year-old man with a history of systemic hypertension, type 2 diabetes mellitus, and hyperuricemia, who was seen for a 2-year history of asymptomatic but progressive lesions that had appeared on the left lower limb. One year before consultation, he had been diagnosed with monoclonal gammopathy of undetermined significance (MGUS), after detecting an IgG(κ) paraprotein in the serum with no evidence of multiple myeloma in the bone marrow study. Physical examination revealed a plaque with an area of ecchymotic appearance and several hard, reddish-orange nodules with an ulcerated surface in the pretibial region of the left leg (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Histopathological examination showed deposits of an amorphous eosinophilic material diffusely distributed through the papillary and reticular dermis, extending into the subcutaneous tissue, accompanied by a dense infiltrate of plasma cells (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). When stained with thioflavin, the deposits fluoresced under ultraviolet light. Immunohistochemical staining demonstrated λ light-chain restriction in the majority of the plasma cells. These findings suggested a possible diagnosis of PLCNA, although systemic amyloidosis could not be ruled out.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Laboratory tests including complete blood count, biochemistry tests, and liver and kidney profiles were normal. Immunoglobulin concentrations were normal, although the IgG value was at the upper limit of normal: 1590<span class="elsevierStyleHsp" style=""></span>mg/dL (normal range, 700–1600<span class="elsevierStyleHsp" style=""></span>mg/dL). Immunoelectrophoresis showed a monoclonal IgG(κ) band with oligoclonal λ bands in the serum, but was normal in the urine. No amyloid deposits were observed in a biopsy of the abdominal fat. After 18 months of follow-up, the patient has not shown evidence of progression to myeloma or systemic amyloidosis.</p><p id="par0025" class="elsevierStylePara elsevierViewall">PLCNA is a disease that occurs predominantly in women, with a mean age at diagnosis of 60 years.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> It has sometimes been described in association with systemic diseases such as Sjögren syndrome,<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> diabetes mellitus, or CREST syndrome.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> It manifests as waxy nodules that are usually solitary or localized or, less commonly, disseminated.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> It tends to affect acral areas, the most common site being the lower limbs, followed by the face and trunk. Histopathological findings are indistinguishable from those of primary systemic amyloidosis or myeloma-associated amyloidosis.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Immunohistochemical staining may show the presence of immunoglobulin light chains in the amyloid or in the cytoplasm of plasma cells.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Based on the demonstration of clonality of the plasma cell infiltrate in the skin using gene rearrangement techniques and on the absence of clonal rearrangement in bone marrow, some authors have suggested that PLCNA should be considered an extramedullary plasmacytoma that produces localized amyloid deposition.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> Extramedullary plasmacytomas are plasma cell neoplasms that arise in any organ except bone marrow; they are able to produce an M-component in the serum in up to 20% of cases.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> Extramedullary plasmacytomas that initially do not produce the M-component in serum progress to multiple myeloma in 20% of cases.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">In our patient, there was a monoclonal infiltrate of plasma cells that synthesized λ chains and produced the amyloid deposits in the skin, and a finding of a monoclonal IgG(κ) band and oligoclonal λ bands in the serum. This suggests a biclonal gammopathy (the presence of 2 monoclonal components in serum), which has been observed in approximately 3% of MGUS.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The appearance of paraproteinemia in cases of PLCNA has rarely been described in the literature, though it should always be investigated at the initial assessment. In the cases in which it has been observed, it was generally associated with an advanced stage of the disease, and may indicate progression to systemic amyloidosis.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">The risk of progression to systemic amyloidosis in PLCNA has been well defined by several authors. Since 1970, the risk has been established at around 50%. However, in 2001, Woollons and Black<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> observed only 1 case of progression to systemic amyloidosis in a series of 15 patients with PLCNA, in addition to high immunoglobulin concentrations in 40% of cases. In 2003, Moon et al.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> obtained similar results after 10 years of follow-up in 16 patients with PLCNA and reported progression in the only patient presenting with paraproteinemia at the time of diagnosis.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Despite its generally benign course, it is important to follow-up these patients closely, especially in cases associated with paraproteinemia, to monitor for possible progression to systemic amyloidosis and to exclude plasma cell dyscrasias.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara">Please cite this article as: Villar M, et al. Amiloidosis nodular primaria cutánea localizada en un pacinete con paraproteinemia. Actas Dermosifiliogr. 2012;103:161–2.</p>" ] ] "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" => 1020 "Ancho" => 1587 "Tamanyo" => 132614 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Well-defined, shiny, reddish-orange plaque with an area of ecchymotic appearance, and 2 firm nodules covered by reddish-orange skin except for one area of ulceration, located in the left lower limb.</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" => 1695 "Ancho" => 2252 "Tamanyo" => 820112 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Deposits of large amounts of amyloid in the dermis, mainly around vessels and adnexa, accompanied by a dense plasma cell infiltrate.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Primary cutaneous nodular amyloidosis: case report and review of the literature" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "M.N. 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Year/Month | Html | Total | |
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2024 November | 5 | 10 | 15 |
2024 October | 106 | 53 | 159 |
2024 September | 83 | 28 | 111 |
2024 August | 115 | 64 | 179 |
2024 July | 101 | 53 | 154 |
2024 June | 93 | 52 | 145 |
2024 May | 123 | 39 | 162 |
2024 April | 104 | 33 | 137 |
2024 March | 73 | 28 | 101 |
2024 February | 79 | 39 | 118 |
2024 January | 72 | 42 | 114 |
2023 December | 64 | 20 | 84 |
2023 November | 79 | 32 | 111 |
2023 October | 80 | 22 | 102 |
2023 September | 77 | 38 | 115 |
2023 August | 52 | 13 | 65 |
2023 July | 113 | 44 | 157 |
2023 June | 67 | 27 | 94 |
2023 May | 134 | 35 | 169 |
2023 April | 87 | 30 | 117 |
2023 March | 66 | 30 | 96 |
2023 February | 69 | 30 | 99 |
2023 January | 62 | 32 | 94 |
2022 December | 64 | 53 | 117 |
2022 November | 37 | 26 | 63 |
2022 October | 25 | 22 | 47 |
2022 September | 32 | 32 | 64 |
2022 August | 27 | 30 | 57 |
2022 July | 27 | 37 | 64 |
2022 June | 27 | 32 | 59 |
2022 May | 61 | 35 | 96 |
2022 April | 82 | 49 | 131 |
2022 March | 89 | 56 | 145 |
2022 February | 90 | 38 | 128 |
2022 January | 78 | 48 | 126 |
2021 December | 64 | 45 | 109 |
2021 November | 77 | 58 | 135 |
2021 October | 60 | 54 | 114 |
2021 September | 59 | 39 | 98 |
2021 August | 67 | 40 | 107 |
2021 July | 44 | 26 | 70 |
2021 June | 52 | 29 | 81 |
2021 May | 52 | 45 | 97 |
2021 April | 126 | 71 | 197 |
2021 March | 71 | 26 | 97 |
2021 February | 59 | 26 | 85 |
2021 January | 47 | 19 | 66 |
2020 December | 39 | 21 | 60 |
2020 November | 42 | 18 | 60 |
2020 October | 37 | 15 | 52 |
2020 September | 41 | 22 | 63 |
2020 August | 37 | 11 | 48 |
2020 July | 19 | 19 | 38 |
2020 June | 51 | 18 | 69 |
2020 May | 40 | 18 | 58 |
2020 April | 32 | 19 | 51 |
2020 March | 37 | 14 | 51 |
2020 February | 3 | 2 | 5 |
2019 December | 2 | 3 | 5 |
2019 November | 0 | 1 | 1 |
2019 September | 2 | 0 | 2 |
2019 August | 0 | 5 | 5 |
2019 July | 0 | 6 | 6 |
2019 June | 1 | 5 | 6 |
2019 May | 1 | 4 | 5 |
2019 April | 0 | 9 | 9 |
2019 March | 2 | 5 | 7 |
2019 February | 0 | 4 | 4 |
2019 January | 2 | 0 | 2 |
2018 December | 2 | 5 | 7 |
2018 November | 2 | 0 | 2 |
2018 October | 2 | 0 | 2 |
2018 September | 0 | 7 | 7 |
2018 August | 0 | 9 | 9 |
2018 July | 0 | 7 | 7 |
2018 June | 0 | 7 | 7 |
2018 May | 0 | 2 | 2 |
2018 April | 0 | 4 | 4 |
2018 March | 2 | 1 | 3 |
2018 February | 148 | 8 | 156 |
2018 January | 97 | 7 | 104 |
2017 December | 169 | 11 | 180 |
2017 November | 60 | 4 | 64 |
2017 October | 49 | 4 | 53 |
2017 September | 61 | 7 | 68 |
2017 August | 113 | 6 | 119 |
2017 July | 92 | 7 | 99 |
2017 June | 88 | 6 | 94 |
2017 May | 69 | 6 | 75 |
2017 April | 56 | 5 | 61 |
2017 March | 61 | 2 | 63 |
2017 February | 82 | 7 | 89 |
2017 January | 65 | 4 | 69 |
2016 December | 72 | 13 | 85 |
2016 November | 78 | 10 | 88 |
2016 October | 86 | 14 | 100 |
2016 September | 66 | 15 | 81 |
2016 August | 73 | 4 | 77 |
2016 July | 48 | 6 | 54 |
2016 June | 6 | 11 | 17 |
2016 May | 5 | 7 | 12 |
2016 April | 3 | 17 | 20 |
2016 March | 12 | 1 | 13 |
2016 February | 8 | 1 | 9 |
2016 January | 8 | 2 | 10 |
2015 December | 9 | 0 | 9 |
2015 November | 26 | 3 | 29 |
2015 October | 30 | 3 | 33 |
2015 September | 16 | 9 | 25 |
2015 August | 11 | 5 | 16 |
2015 July | 45 | 13 | 58 |
2015 June | 43 | 40 | 83 |
2015 May | 65 | 7 | 72 |
2015 April | 73 | 8 | 81 |
2015 March | 48 | 7 | 55 |
2015 February | 43 | 9 | 52 |
2015 January | 55 | 9 | 64 |
2014 December | 34 | 9 | 43 |
2014 November | 46 | 9 | 55 |
2014 October | 66 | 14 | 80 |
2014 September | 39 | 16 | 55 |
2014 August | 55 | 11 | 66 |
2014 July | 64 | 19 | 83 |
2014 June | 85 | 14 | 99 |
2014 May | 77 | 13 | 90 |
2014 April | 60 | 12 | 72 |
2014 March | 63 | 17 | 80 |
2014 February | 22 | 10 | 32 |
2014 January | 26 | 6 | 32 |
2013 December | 25 | 5 | 30 |
2013 November | 24 | 12 | 36 |
2013 October | 12 | 4 | 16 |
2013 September | 13 | 6 | 19 |
2013 August | 15 | 10 | 25 |
2013 July | 13 | 7 | 20 |
2013 June | 16 | 6 | 22 |
2013 May | 10 | 20 | 30 |
2013 April | 15 | 11 | 26 |
2013 March | 21 | 11 | 32 |
2013 February | 35 | 9 | 44 |
2013 January | 50 | 2 | 52 |
2012 December | 26 | 8 | 34 |
2012 November | 1 | 1 | 2 |
2012 October | 0 | 2 | 2 |