Case for Diagnosis
Bruise-like Spots Appearing Progressively on the Face and Back
Manchas contusiformes de aparición progresiva en la cara y la espalda
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Ruiz-Villaverde, B. Rueda-Villafranca, J.C. Ruiz-Carrascosa" "autores" => array:3 [ 0 => array:4 [ "nombre" => "R." "apellidos" => "Ruiz-Villaverde" "email" => array:1 [ 0 => "ismenios@hotmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "*" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "B." "apellidos" => "Rueda-Villafranca" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "J.C." 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Palpation revealed no locoregional enlarged lymph nodes in the neck, axillae, or groin, nor enlarged liver or spleen.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">A lymphocytic infiltrate was observed throughout the thickness of the dermis, in the form of converging patches with a Grenz band against the epidermis (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). Immune staining was positive for CD45, CD4, CD1a, CD43, CD56, and CD123 (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>), negative for CD20, CD138, CD8, and myeloperoxidase, and weakly positive for CD33. The Ki-67 labeling index was 40%.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">Analyses revealed the following: Hb, 11.9<span class="elsevierStyleHsp" style=""></span>g/dL; l<span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>2180/Ul; platelets, 78.000/Ul; and B2 microglobulin, 6.7 (N<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>5<span class="elsevierStyleHsp" style=""></span>mg/dL). The immune phenotype of the peripheral blood cells was as follows: monocytes, 16.5%; lymphocytes, 28.16%; TCD3+ lymphocytes, 22.5%; CD4+, 15.8%; CD8+, 5.66%; B lymphocytes, 0.6%; NK cells, 4.4%; and neutrophils, 53.88%. All cells were mature CD10+ granulocytes. No blast cells were detected. Fine-needle aspiration of the bone marrow revealed normal bone-marrow cells with erythroid hyperplasia with no evident dysplastic signs of blood disease. Imaging studies produced normal results (abdominal ultrasound, CT scan, and PET-CT).</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">What is your diagnosis?</span><p id="par0025" class="elsevierStylePara elsevierViewall">Diagnosis</p><p id="par0030" class="elsevierStylePara elsevierViewall">Blastic plasmacytoid dendritic cell neoplasm (WHO 2017) or CD4+/CD56+ hematodermal neoplasm.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">Study of disease extension revealed no involvement of the bone marrow. Treatment was instated by protocol with cyclophosphamide, vincristine, doxorubicin, and dexamethasone for frail patients over 45 years of age. The clinical course was favorable after 3 months of treatment and no new lesions appeared. The patient continues in periodic follow-up by the dermatology and hematology departments.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">Different subtypes of lymphoma that express CD56 have emerged in recent years. These include hematodermal CD4+/CD56+ or blastic plasmacytoid dendritic cell neoplasm. This entity was first described by Adachi in 1994.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> In 2001, Chaperot<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> considered plamacytoid dendritic cells to be the precursor cells of this neoplasm and the entity acquired its definitive name in the 2005 WHO-EORTC update.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> It consists of an aggressive hematologic disorder with a high incidence of cutaneous involvement and a major risk of leukemic cell dissemination.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">It typically affects middle-aged and elderly men with no predominance of any race. It rarely presents in children and only 12 pediatric cases have been reported to date.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> It presents clinically as violaceous bruise-like spots or plaques, which are painless in most cases, with a predilection for the torso, although the head and neck may also be involved, as may the limbs. Involvement of the lymph nodes is relatively common (between 40% and 50% of cases), although B symptoms are extraordinarily rare.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">From a histology point of view, this variety of lymphoma does not present epidermotropism. The lymphocytic infiltrate affects the dermis massively and sometimes goes deeper into the subcutaneous cellular tissue. Necrosis and vascular invasion are not usual and mitotic figures tend to be present</p><p id="par0055" class="elsevierStylePara elsevierViewall">The immune-staining profile of most of these tumors is the following: CD4+/CD56+/CD8–/CD7–/CD45+ and negative for B-cell markers and other T cells.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">The differential diagnosis should focus on lymphoproliferative disorders with primary and secondary skin involvement, especially acute leukemia, myelodysplastic syndromes, and lymphoblastic lymphomas and nasal or extranasal NK lymphomas.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Prognosis is poor and the mean survival rate has been estimated in most series consulted to be approximately 14 months. Dissemination to the bone marrow tends to be a rapid process once the skin lesions appear. For this reason, most of these cases must be treated at multidisciplinary centers and with aggressive regimens that consider this entity as equivalent to an acute leukemia. In cases where response is favorable, development of residual hyperpigmentation is typical, as is recurrence if therapy is interrupted. A good response has been reported following use of the combined protocol of chemotherapy and radiation therapy followed by allogenic stem-cell transplant.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a></p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicts of Interest</span><p id="par0070" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:9 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Medical History" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Physical Examination" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Histopathology" ] 3 => array:2 [ "identificador" => "sec0020" "titulo" => "Additional Tests" ] 4 => array:2 [ "identificador" => "sec0025" "titulo" => "What is your diagnosis?" ] 5 => array:2 [ "identificador" => "sec0030" "titulo" => "Clinical Course and Treatment" ] 6 => array:2 [ "identificador" => "sec0035" "titulo" => "Comment" ] 7 => array:2 [ "identificador" => "sec0040" "titulo" => "Conflicts of Interest" ] 8 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2019-02-20" "fechaAceptado" => "2019-05-22" "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Ruiz-Villaverde R, Rueda-Villafranca B, Ruiz-Carrascosa JC. Manchas contusiformes de aparición progresiva en la cara y la espalda. Actas Dermosifiliogr. 2021;112:447–448.</p>" ] ] "multimedia" => array:3 [ 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" => 500 "Tamanyo" => 54473 ] ] ] 1 => array:8 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 450 "Ancho" => 800 "Tamanyo" => 157442 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Hematoxylin–eosin, ×20.</p>" ] ] 2 => array:8 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 449 "Ancho" => 800 "Tamanyo" => 119208 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0010" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A, CD45. B, CD1a. C, CD43. D, CD123. E, CD56. F, CD4 (×2).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:7 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "High expression of CD56 (N-CAM) in a patient with cutaneous CD4-positive lymphoma" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "M. Adachi" 1 => "K. Maeda" 2 => "M. Takekawa" 3 => "Y. Hinoda" 4 => "K. Imai" 5 => "S. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 10 | 9 | 19 |
| 2024 October | 123 | 48 | 171 |
| 2024 September | 131 | 22 | 153 |
| 2024 August | 124 | 57 | 181 |
| 2024 July | 135 | 27 | 162 |
| 2024 June | 114 | 26 | 140 |
| 2024 May | 102 | 24 | 126 |
| 2024 April | 93 | 23 | 116 |
| 2024 March | 88 | 30 | 118 |
| 2024 February | 86 | 24 | 110 |
| 2024 January | 97 | 31 | 128 |
| 2023 December | 75 | 20 | 95 |
| 2023 November | 102 | 21 | 123 |
| 2023 October | 99 | 22 | 121 |
| 2023 September | 58 | 35 | 93 |
| 2023 August | 60 | 11 | 71 |
| 2023 July | 81 | 29 | 110 |
| 2023 June | 56 | 27 | 83 |
| 2023 May | 70 | 26 | 96 |
| 2023 April | 60 | 23 | 83 |
| 2023 March | 57 | 22 | 79 |
| 2023 February | 59 | 22 | 81 |
| 2023 January | 62 | 27 | 89 |
| 2022 December | 40 | 46 | 86 |
| 2022 November | 26 | 35 | 61 |
| 2022 October | 26 | 28 | 54 |
| 2022 September | 21 | 41 | 62 |
| 2022 August | 30 | 43 | 73 |
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| 2022 June | 21 | 38 | 59 |
| 2022 May | 43 | 30 | 73 |
| 2022 April | 36 | 37 | 73 |
| 2022 March | 49 | 65 | 114 |
| 2022 February | 51 | 47 | 98 |
| 2022 January | 33 | 42 | 75 |
| 2021 December | 41 | 38 | 79 |
| 2021 November | 49 | 56 | 105 |
| 2021 October | 45 | 70 | 115 |
| 2021 September | 59 | 45 | 104 |
| 2021 August | 45 | 38 | 83 |
| 2021 July | 30 | 40 | 70 |
| 2021 June | 37 | 45 | 82 |
| 2021 May | 60 | 52 | 112 |
| 2021 April | 83 | 77 | 160 |
| 2021 March | 121 | 48 | 169 |
| 2021 February | 2 | 16 | 18 |
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