Cases and Research Letters
Generalized Eruptive Histiocytosis in a Patient with Chronic Myelomonocytic Leukemia
Histiocitosis eruptiva generalizada en paciente con leucemia mielomonocítica crónica
I. Montero
, E. Gutiérrez-González, M. Ginarte, J. Toribio
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Departamento de Dermatología, Complejo Hospitalario Universitario, Facultad de Medicina, Santiago de Compostela, Spain
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Multiple spotty erythematous plaques on the lower lip, mulberry-like in appearance.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "B. Di MartinoOrtiz, M.L. Rodríguez-Oviedo, M. Rodríguez-Masi" "autores" => array:3 [ 0 => array:2 [ "nombre" => "B." "apellidos" => "Di MartinoOrtiz" ] 1 => array:2 [ "nombre" => "M.L." "apellidos" => "Rodríguez-Oviedo" ] 2 => array:2 [ "nombre" => "M." 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Aportación de un nuevo caso" ] ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "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" => 900 "Ancho" => 1750 "Tamanyo" => 186693 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Infiltrated plaques with orange-peel skin. A, Deltoid area. B, Lower back.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "M. Castellanos-González, G. Petiti-Martín, C. Postigo, J.L. Rodriguez-Peralto" "autores" => array:4 [ 0 => array:2 [ "nombre" => "M." "apellidos" => "Castellanos-González" ] 1 => array:2 [ "nombre" => "G." "apellidos" => "Petiti-Martín" ] 2 => array:2 [ "nombre" => "C." 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Montero, E. Gutiérrez-González, M. Ginarte, J. Toribio" "autores" => array:4 [ 0 => array:4 [ "nombre" => "I." "apellidos" => "Montero" "email" => array:1 [ 0 => "iriamontero@hotmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">¿</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "E." "apellidos" => "Gutiérrez-González" ] 2 => array:2 [ "nombre" => "M." "apellidos" => "Ginarte" ] 3 => array:2 [ "nombre" => "J." "apellidos" => "Toribio" ] ] "afiliaciones" => array:1 [ 0 => array:1 [ "entidad" => "Departamento de Dermatología, Complejo Hospitalario Universitario, Facultad de Medicina, Santiago de Compostela, Spain" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Histiocitosis eruptiva generalizada en paciente con leucemia mielomonocítica crónica" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0020" "etiqueta" => "Figure 4" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr4.jpeg" "Alto" => 629 "Ancho" => 951 "Tamanyo" => 180988 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Infiltrate consisting of histiocytes, some of which are vacuolated, lymphocytes, and some multinucleated giant cells (hematoxylin-eosin, original magnification <span class="elsevierStyleHsp" style=""></span>×250).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">An 80-year-old man visited our department with asymptomatic skin lesions that had appeared 2 months earlier. The patient stated that he was not receiving any regular treatments and had not been administered new drugs; he reported no fever or other symptoms and did not recall having been bitten or stung.</p><p id="par0010" class="elsevierStylePara elsevierViewall">Physical examination revealed erythematous papules on the upper part of the torso; the papules measured approximately 3<span class="elsevierStyleHsp" style=""></span>mm in diameter and were firm with a depressed center (<a class="elsevierStyleCrossRefs" href="#fig0005">Figs. 1 and 2</a>). Some of the papules had coalesced to form plaques. The mucosa, palms, and soles were not affected.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">A skin biopsy revealed a dense infiltrate in the papillary and middle dermis, consisting mainly of histiocytes, some of which were vacuolated, lymphocytes, and some multinucleated giant cells (<a class="elsevierStyleCrossRefs" href="#fig0015">Figs. 3 and 4</a>). Immune staining was positive for CD68 and negative for S100, CD1a, and factor XIIIa.</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Laboratory tests and x-ray studies showed only monocytosis, which had already been diagnosed and was being followed up by the hematology department after a diagnosis of chronic myelomonocytic leukemia with limited bone marrow dysplasia and a normal karyotype.</p><p id="par0025" class="elsevierStylePara elsevierViewall">The patient was diagnosed with generalized eruptive histiocytosis and a wait-and-see approach was adopted; the lesions disappeared 6 months after diagnosis. The chronic myelomonocytic leukemia has remained stable to date.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Histiocytosis comprises a rare, heterogeneous group of diseases characterized by the proliferation of histiocytes. Most authors continue to use the traditional classification of the entity into Langerhans cell histiocytosis, type I histiocytosis or histiocytosis X, and non-Langerhans cell, type II, or non-X histiocytosis.</p><p id="par0035" class="elsevierStylePara elsevierViewall">The group of non-Langerhans cell histiocytoses includes multiple diseases, such as generalized eruptive histiocytosis. Some authors maintain that these are not independent diseases but are different stages in the course of the same disease, and that generalized eruptive histiocytosis is the initial stage of the process, with subsequent development of xanthomatous lesions.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">In 1963, Winkelmann and Muller<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> described generalized eruptive histiocytosis as a rare condition characterized by multiple noncoalescing, symmetric erythematous papules distributed on the torso and limbs. The disease is not accompanied by extracutaneous involvement and histology shows a monomorphic infiltrate consisting predominantly of histiocytes that are negative for S100 and lipids. The disease has a benign course and mainly affects middle-aged patients. It tends to remit spontaneously and, although the cause has not been established, some authors maintain that it is produced by the apoptosis mechanism.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">In our patient, the differential diagnosis included generalized granuloma anulare, which was ruled out, and immune staining indicated a diagnosis of a type of non-Langerhans cell histiocytosis. The clinical appearance of the lesions, their location, and the patient's age at onset ruled out other types non-Langerhans cell histiocytosis, such as benign cephalic histiocytosis, multicentric reticulohistiocytosis, and progressive nodular histiocytoma. The principal differential diagnosis includes other forms of xanthomatous non-Langerhans cell histiocytosis, particularly adult papular xanthoma. Adult papular xanthoma may be a later stage in the development of the disease, in which the clinical lesions take on a yellowish color and are characterized histologically by a greater number of xanthomatous cells.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">A wait-and-see approach is usually adopted, as the condition tends to resolve spontaneously within a few months. Treatment should be considered when remission does not occur and good results have been reported for systemic treatment with corticosteroids, hydroxychloroquine, and thalidomide,<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> with psoralen-UVA<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> or even cryotherapy, peeling, or carbon dioxide laser treatment in localized lesions.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Our patient presented 3 unique aspects: coalescence of the lesions, negative immune staining for factor XIIIa, and association with chronic myelomonocytic leukemia.</p><p id="par0060" class="elsevierStylePara elsevierViewall">Coalescence of the lesions has been reported in the late stage of this disease. Klemke et al.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> linked this clinical sign in this type of histiocytosis with acute monocytic leukemia and with a worse prognosis for the disease. Hence, the coalescence of the lesions in our patient and the myelomonocytic leukemia support this observation.</p><p id="par0065" class="elsevierStylePara elsevierViewall">Another characteristic of our patient is the negative immune staining for factor XIIIa, which is usually positive in generalized eruptive histiocytosis; 2 other cases have been reported in the literature with this characteristic.<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6,8</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">In general, non-Langerhans cell histiocytosis presents a benign course in healthy patients and does not usually appear in association with malignant diseases. However, some types of non-Langerhans cell histiocytosis, such as progressive nodular histiocytosis and juvenile xanthogranuloma, have been described in association with hematologic disorders.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> To date, 2 cases have been reported in the literature of generalized eruptive histiocytosis associated with acute monocytic leukemia,<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">7,10</span></a> but none have been described in association with chronic myelomonocytic leukemia, as was the case with our patient.</p><p id="par0075" class="elsevierStylePara elsevierViewall">In conclusion, we report what we believe to be the first case of a patient with generalized eruptive histiocytosis associated with chronic myelomonocytic leukemia. In our opinion, given the frequency of hematologic disorders in patients with non-Langerhans cell histiocytosis, further study of this association is needed in patients with these signs and symptoms, and more specifically, in patients with generalized eruptive histiocytosis with coalescing lesions.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara">Please cite this article as: Montero I, Gutiérrez-González R, Ginarte M, Toribio J. Histiocitosis eruptive generalizada en paciente con leukemia mielomonocítica crónica. Actas Dermosifiliogr. 2012;103:643–644.</p>" ] ] "multimedia" => array:4 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1273 "Ancho" => 951 "Tamanyo" => 167067 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Multiple erythematous papules distributed on the patient's torso; some of the papules have coalesced to form small plaques.</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" => 711 "Ancho" => 951 "Tamanyo" => 124610 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Detail of the lesions, showing millimeter-sized umbilicated papules.</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 631 "Ancho" => 951 "Tamanyo" => 237561 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Dense infiltrate in the papillary and middle dermis (hematoxylin-eosin, original magnification <span class="elsevierStyleHsp" style=""></span>×100).</p>" ] ] 3 => array:7 [ "identificador" => "fig0020" "etiqueta" => "Figure 4" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr4.jpeg" "Alto" => 629 "Ancho" => 951 "Tamanyo" => 180988 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Infiltrate consisting of histiocytes, some of which are vacuolated, lymphocytes, and some multinucleated giant cells (hematoxylin-eosin, original magnification <span class="elsevierStyleHsp" style=""></span>×250).</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" => "Benign non-X histiocytosis: a unique case bridging several of the non-X histiocytic syndromes" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "B.M. 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| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 16 | 9 | 25 |
| 2024 Octubre | 80 | 40 | 120 |
| 2024 Septiembre | 101 | 29 | 130 |
| 2024 Agosto | 113 | 61 | 174 |
| 2024 Julio | 94 | 31 | 125 |
| 2024 Junio | 102 | 34 | 136 |
| 2024 Mayo | 81 | 34 | 115 |
| 2024 Abril | 69 | 26 | 95 |
| 2024 Marzo | 94 | 36 | 130 |
| 2024 Febrero | 80 | 32 | 112 |
| 2024 Enero | 73 | 35 | 108 |
| 2023 Diciembre | 78 | 19 | 97 |
| 2023 Noviembre | 68 | 25 | 93 |
| 2023 Octubre | 60 | 16 | 76 |
| 2023 Septiembre | 71 | 29 | 100 |
| 2023 Agosto | 63 | 34 | 97 |
| 2023 Julio | 79 | 42 | 121 |
| 2023 Junio | 69 | 29 | 98 |
| 2023 Mayo | 90 | 32 | 122 |
| 2023 Abril | 63 | 32 | 95 |
| 2023 Marzo | 39 | 26 | 65 |
| 2023 Febrero | 50 | 24 | 74 |
| 2023 Enero | 58 | 42 | 100 |
| 2022 Diciembre | 48 | 36 | 84 |
| 2022 Noviembre | 25 | 19 | 44 |
| 2022 Octubre | 38 | 19 | 57 |
| 2022 Septiembre | 25 | 24 | 49 |
| 2022 Agosto | 33 | 36 | 69 |
| 2022 Julio | 29 | 28 | 57 |
| 2022 Junio | 30 | 24 | 54 |
| 2022 Mayo | 76 | 37 | 113 |
| 2022 Abril | 100 | 52 | 152 |
| 2022 Marzo | 80 | 64 | 144 |
| 2022 Febrero | 49 | 29 | 78 |
| 2022 Enero | 50 | 39 | 89 |
| 2021 Diciembre | 56 | 29 | 85 |
| 2021 Noviembre | 52 | 53 | 105 |
| 2021 Octubre | 47 | 51 | 98 |
| 2021 Septiembre | 45 | 37 | 82 |
| 2021 Agosto | 53 | 24 | 77 |
| 2021 Julio | 30 | 15 | 45 |
| 2021 Junio | 50 | 34 | 84 |
| 2021 Mayo | 41 | 25 | 66 |
| 2021 Abril | 65 | 63 | 128 |
| 2021 Marzo | 69 | 23 | 92 |
| 2021 Febrero | 68 | 22 | 90 |
| 2021 Enero | 36 | 25 | 61 |
| 2020 Diciembre | 45 | 12 | 57 |
| 2020 Noviembre | 29 | 8 | 37 |
| 2020 Octubre | 26 | 4 | 30 |
| 2020 Septiembre | 21 | 8 | 29 |
| 2020 Agosto | 18 | 16 | 34 |
| 2020 Julio | 35 | 12 | 47 |
| 2020 Junio | 37 | 24 | 61 |
| 2020 Mayo | 36 | 22 | 58 |
| 2020 Abril | 29 | 17 | 46 |
| 2020 Marzo | 40 | 25 | 65 |
| 2020 Febrero | 5 | 2 | 7 |
| 2020 Enero | 3 | 3 | 6 |
| 2019 Diciembre | 6 | 2 | 8 |
| 2019 Noviembre | 4 | 5 | 9 |
| 2019 Octubre | 0 | 8 | 8 |
| 2019 Septiembre | 0 | 2 | 2 |
| 2019 Agosto | 6 | 4 | 10 |
| 2019 Julio | 4 | 3 | 7 |
| 2019 Junio | 4 | 1 | 5 |
| 2019 Mayo | 4 | 16 | 20 |
| 2019 Abril | 2 | 3 | 5 |
| 2019 Marzo | 2 | 6 | 8 |
| 2019 Enero | 3 | 1 | 4 |
| 2018 Diciembre | 2 | 0 | 2 |
| 2018 Noviembre | 1 | 1 | 2 |
| 2018 Octubre | 4 | 0 | 4 |
| 2018 Septiembre | 4 | 0 | 4 |
| 2018 Junio | 0 | 1 | 1 |
| 2018 Mayo | 0 | 6 | 6 |
| 2018 Marzo | 8 | 4 | 12 |
| 2018 Febrero | 42 | 4 | 46 |
| 2018 Enero | 58 | 6 | 64 |
| 2017 Diciembre | 55 | 8 | 63 |
| 2017 Noviembre | 49 | 7 | 56 |
| 2017 Octubre | 39 | 4 | 43 |
| 2017 Septiembre | 40 | 11 | 51 |
| 2017 Agosto | 63 | 8 | 71 |
| 2017 Julio | 60 | 5 | 65 |
| 2017 Junio | 66 | 22 | 88 |
| 2017 Mayo | 68 | 15 | 83 |
| 2017 Abril | 72 | 10 | 82 |
| 2017 Marzo | 56 | 21 | 77 |
| 2017 Febrero | 41 | 19 | 60 |
| 2017 Enero | 51 | 12 | 63 |
| 2016 Diciembre | 57 | 16 | 73 |
| 2016 Noviembre | 70 | 12 | 82 |
| 2016 Octubre | 108 | 20 | 128 |
| 2016 Septiembre | 130 | 10 | 140 |
| 2016 Agosto | 54 | 9 | 63 |
| 2016 Julio | 34 | 12 | 46 |
| 2016 Junio | 8 | 11 | 19 |
| 2016 Mayo | 4 | 13 | 17 |
| 2016 Abril | 7 | 1 | 8 |
| 2016 Marzo | 13 | 1 | 14 |
| 2016 Febrero | 5 | 5 | 10 |
| 2016 Enero | 8 | 1 | 9 |
| 2015 Diciembre | 6 | 16 | 22 |
| 2015 Noviembre | 20 | 3 | 23 |
| 2015 Octubre | 18 | 22 | 40 |
| 2015 Septiembre | 4 | 7 | 11 |
| 2015 Agosto | 17 | 1 | 18 |
| 2015 Julio | 81 | 11 | 92 |
| 2015 Junio | 61 | 7 | 68 |
| 2015 Mayo | 70 | 16 | 86 |
| 2015 Abril | 74 | 18 | 92 |
| 2015 Marzo | 72 | 5 | 77 |
| 2015 Febrero | 63 | 7 | 70 |
| 2015 Enero | 70 | 13 | 83 |
| 2014 Diciembre | 52 | 8 | 60 |
| 2014 Noviembre | 54 | 11 | 65 |
| 2014 Octubre | 113 | 14 | 127 |
| 2014 Septiembre | 76 | 14 | 90 |
| 2014 Agosto | 40 | 5 | 45 |
| 2014 Julio | 40 | 5 | 45 |
| 2014 Junio | 44 | 4 | 48 |
| 2014 Mayo | 49 | 5 | 54 |
| 2014 Abril | 45 | 5 | 50 |
| 2014 Marzo | 53 | 7 | 60 |
| 2014 Febrero | 43 | 5 | 48 |
| 2014 Enero | 53 | 11 | 64 |
| 2013 Diciembre | 49 | 7 | 56 |
| 2013 Noviembre | 46 | 10 | 56 |
| 2013 Octubre | 36 | 7 | 43 |
| 2013 Septiembre | 25 | 3 | 28 |
| 2013 Agosto | 15 | 11 | 26 |
| 2013 Julio | 12 | 5 | 17 |
| 2013 Junio | 15 | 8 | 23 |
| 2013 Mayo | 16 | 10 | 26 |
| 2013 Abril | 14 | 7 | 21 |
| 2013 Marzo | 15 | 7 | 22 |
| 2013 Febrero | 38 | 4 | 42 |
| 2013 Enero | 58 | 5 | 63 |
| 2012 Diciembre | 29 | 6 | 35 |
| 2012 Noviembre | 0 | 1 | 1 |
| 2012 Octubre | 0 | 1 | 1 |
| 2012 Septiembre | 1 | 1 | 2 |
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