Case and Research Letter
Ultrasound features of Primary Cutaneous CD4+ Small/Medium-Sized T-Cell Lymphoproliferative Disorder
Ecografía del síndrome linfoproliferativo primario cutáneo T CD4+ de célula pequeña/mediana
E. Rodríguez-Lomba
, I. Molina-López, A. Pulido-Pérez, C. Ciudad-Blanco
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Servicio de Dermatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain
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Rodríguez-Lomba, I. Molina-López, A. Pulido-Pérez, C. Ciudad-Blanco" "autores" => array:4 [ 0 => array:4 [ "nombre" => "E." "apellidos" => "Rodríguez-Lomba" "email" => array:1 [ 0 => "enriquerlomba@outlook.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "I." "apellidos" => "Molina-López" ] 2 => array:2 [ "nombre" => "A." "apellidos" => "Pulido-Pérez" ] 3 => array:2 [ "nombre" => "C." "apellidos" => "Ciudad-Blanco" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Dermatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Ecografía del síndrome linfoproliferativo primario cutáneo T CD4+ de célula pequeña/mediana" ] ] "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" => 1630 "Ancho" => 1167 "Tamanyo" => 184892 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Erythematous-violaceous subcutaneous nodule of 2<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>1.5<span class="elsevierStyleHsp" style=""></span>cm in the right scapular region.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Primary cutaneous lymphomas have a low incidence in the general population. The clinical presentation is very variable, often making the diagnosis unexpected for the dermatologist. Although the diagnosis of these lesions always requires histopathologic confirmation, skin ultrasound is an additional tool that can be helpful for the initial diagnosis of malignancy. We present a case of primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (PCSM-TCLD) and its ultrasound findings.</p><p id="par0010" class="elsevierStylePara elsevierViewall">A 55-year-old woman with a past history of hypothyroidism and dyslipidemia came to the emergency department for a pruritic lesion in the right scapular region; the lesion had grown rapidly in the previous 2 months. She reported no fever, asthenia, or weight loss, and she denied any previous lesions or trauma.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Physical examination revealed a solid, tender erythematous-violaceous nodule of 2<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>1.5<span class="elsevierStyleHsp" style=""></span>cm in the right scapular region (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). The nodule was not adherent to deeper planes. A surgical drainage-aspiration procedure performed in the emergency department excluded the presence of fluid collections. There were no palpable lymph nodes or hepatosplenomegaly.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Skin ultrasound of the lesion was performed in dermatology outpatient clinic. Examination in B mode (18<span class="elsevierStyleHsp" style=""></span>MHz probe, MyLab 25 Gold, Esaote, Italy) revealed a dermal nodule of 20<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>15<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>11<span class="elsevierStyleHsp" style=""></span>mm, with deep extension into the subcutaneous cellular tissue. The nodule had a heterogeneous content, was not encapsulated, and had poorly defined borders (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>A). The superficial or dermal plane of the lesion and its deeper or hypodermal plane were noticeably more hypoechoic than the intermediate plane that separated them, and contained hypoechoic tubular tracts. Color Doppler examination revealed the presence of extensive neovascularization, with large vessels within the tumor (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>B).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">These ultrasound findings supported the clinical suspicion of malignancy, and a 4<span class="elsevierStyleHsp" style=""></span>mm punch biopsy was therefore performed for histopathology study. Analysis of the sample showed a dense perivascular and periadnexal interstitial lymphocytic infiltrate that extended into the hypodermis and did not affect the epidermis; the infiltrate was formed of small/medium-sized lymphocytes with mild-to-moderate pleomorphism (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>, A and B). Immunohistochemistry was positive for CD2, CD3, CD4, CD5, and BF1 and negative for CD8, CD30, granzyme, perforin, CD56, CD57, CD20, and CD79. Molecular analysis detected a monoclonal rearrangement of the T-cell receptor gene that, together with the previous findings, was consistent with a diagnosis of PCSM-TCLD.</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">No significant alterations were detected in the metastatic work-up with cervico-thoraco-abdominopelvic computed tomography and a blood test including lymphocyte subpopulations, lactate dehydrogenase, and β2-microglobulin. The patient was offered radiotherapy, which achieved a good response, and there has been no recurrence of the disease.</p><p id="par0035" class="elsevierStylePara elsevierViewall">Primary cutaneous CD4+ small/medium T-cell lymphoma was a provisional entity in the 2008 World Health Organization classification of hematologic tumors.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">1</span></a> Because of its uncertain malignant potential, the term lymphoproliferative disorder has been recommended instead of lymphoma in the more recent 2016 classification.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">2</span></a> The disease typically presents clinically as an erythematous-violaceous plaque or nodule of firm consistency, usually localized on the trunk or face. It is characterized histologically by a diffuse or nodular dermal infiltrate of atypical CD4+ small- and medium-sized lymphocytes, as in the case described. Although the prognosis of these lesions is usually excellent,<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">3,4</span></a> aggressive forms have very rarely been described.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">5</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The widespread incorporation of skin ultrasound into dermatology outpatient clinics has led to the recent development of numerous applications, from the study of inflammatory diseases to definition of the ultrasound characteristics of a number of malignant tumors.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">6</span></a> However, little has been published on the ultrasound findings in cutaneous lymphoma.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">7</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Taking into account the clinical and histopathologic variability of PCSM-TCLD,<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">5</span></a> certain diversity is to be expected in the ultrasound findings. Using studies performed on soft-tissue lymphomas as reference,<a class="elsevierStyleCrossRefs" href="#bib0095"><span class="elsevierStyleSup">8–11</span></a> it can be seen that a diffuse infiltration of atypical lymphocytes usually produces an ultrasound image with hypoechoic and heterogeneous areas with poorly defined borders, as was observed in our patient. The presence of an intense neoangiogenesis on color Doppler appears to be a characteristic finding in soft-tissue lymphomas, and is typically more prominent than in other malignant tumors.<a class="elsevierStyleCrossRefs" href="#bib0100"><span class="elsevierStyleSup">9,10</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">The sonographic differentiation between skin metastases and other tumors can be complicated, but carries important prognostic implications for the patient. Such lesions often present clinical and ultrasound features similar to those described, and this must be taken into account.</p><p id="par0055" class="elsevierStylePara elsevierViewall">In summary, we have presented a case of PCSM-TCLD and its ultrasound findings. These findings helped to orient the initial suspicion of malignancy in a clinically nonspecific lesion. We hope that progressive advances in cutaneous ultrasonography will enable us to better define the diagnosis of this type of tumors.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of Interest</span><p id="par0060" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conflicts of Interest" ] 1 => 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: Rodríguez-Lomba E, Molina-López I, Pulido-Pérez A, Ciudad-Blanco C. Ecografía del síndrome linfoproliferativo primario cutáneo T CD4+ de célula pequeña/mediana. Actas Dermosifiliogr. 2017;108:792–794.</p>" ] ] "multimedia" => array:3 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1630 "Ancho" => 1167 "Tamanyo" => 184892 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Erythematous-violaceous subcutaneous nodule of 2<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>1.5<span class="elsevierStyleHsp" style=""></span>cm in the right scapular region.</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" => 2424 "Ancho" => 1668 "Tamanyo" => 321137 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A, B-mode ultrasound, 18<span class="elsevierStyleHsp" style=""></span>MHz. Dermal nodule with extension into the subcutaneous cellular tissue, with a heterogeneous content and poorly defined borders. Lower echogenicity of the superficial and deep planes compared with the intermediate plane. B, Color Doppler ultrasound showing large-caliber vessels within the tumor.</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" => 1243 "Ancho" => 2527 "Tamanyo" => 899595 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Perivascular and periadnexal interstitial lymphocytic infiltrate that extends into the hypodermis and respects the epidermis. The infiltrate is formed of small/medium-sized lymphocytes with mild-to-moderate pleomorphism. A, Hematoxylin and eosin, original magnification x10. B, Hematoxylin and eosin, original magnification x40.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:11 [ 0 => array:3 [ "identificador" => "bib0060" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "World Health Organization classification of tumours of haematopoietic and lymphoid tissues" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "S.H. Swerdlow" 1 => "E. Campo" 2 => "N.L. Harris" 3 => "E.S. Jaffe" 4 => "S. Pileri" 5 => "H. 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| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 8 | 17 | 25 |
| 2024 Octubre | 84 | 40 | 124 |
| 2024 Septiembre | 97 | 33 | 130 |
| 2024 Agosto | 127 | 85 | 212 |
| 2024 Julio | 91 | 49 | 140 |
| 2024 Junio | 79 | 60 | 139 |
| 2024 Mayo | 60 | 47 | 107 |
| 2024 Abril | 77 | 27 | 104 |
| 2024 Marzo | 68 | 25 | 93 |
| 2024 Febrero | 57 | 35 | 92 |
| 2024 Enero | 52 | 42 | 94 |
| 2023 Diciembre | 58 | 14 | 72 |
| 2023 Noviembre | 88 | 27 | 115 |
| 2023 Octubre | 68 | 24 | 92 |
| 2023 Septiembre | 58 | 26 | 84 |
| 2023 Agosto | 45 | 12 | 57 |
| 2023 Julio | 66 | 32 | 98 |
| 2023 Junio | 71 | 25 | 96 |
| 2023 Mayo | 72 | 22 | 94 |
| 2023 Abril | 78 | 19 | 97 |
| 2023 Marzo | 64 | 18 | 82 |
| 2023 Febrero | 62 | 16 | 78 |
| 2023 Enero | 59 | 30 | 89 |
| 2022 Diciembre | 82 | 35 | 117 |
| 2022 Noviembre | 30 | 28 | 58 |
| 2022 Octubre | 34 | 20 | 54 |
| 2022 Septiembre | 41 | 45 | 86 |
| 2022 Agosto | 40 | 26 | 66 |
| 2022 Julio | 33 | 42 | 75 |
| 2022 Junio | 27 | 22 | 49 |
| 2022 Mayo | 60 | 36 | 96 |
| 2022 Abril | 66 | 30 | 96 |
| 2022 Marzo | 57 | 53 | 110 |
| 2022 Febrero | 42 | 27 | 69 |
| 2022 Enero | 89 | 33 | 122 |
| 2021 Diciembre | 63 | 41 | 104 |
| 2021 Noviembre | 65 | 37 | 102 |
| 2021 Octubre | 56 | 55 | 111 |
| 2021 Septiembre | 53 | 27 | 80 |
| 2021 Agosto | 35 | 31 | 66 |
| 2021 Julio | 53 | 30 | 83 |
| 2021 Junio | 41 | 23 | 64 |
| 2021 Mayo | 41 | 41 | 82 |
| 2021 Abril | 101 | 79 | 180 |
| 2021 Marzo | 78 | 38 | 116 |
| 2021 Febrero | 72 | 28 | 100 |
| 2021 Enero | 57 | 18 | 75 |
| 2020 Diciembre | 49 | 17 | 66 |
| 2020 Noviembre | 42 | 14 | 56 |
| 2020 Octubre | 29 | 14 | 43 |
| 2020 Septiembre | 52 | 24 | 76 |
| 2020 Agosto | 27 | 23 | 50 |
| 2020 Julio | 37 | 18 | 55 |
| 2020 Junio | 40 | 31 | 71 |
| 2020 Mayo | 28 | 25 | 53 |
| 2020 Abril | 30 | 15 | 45 |
| 2020 Marzo | 40 | 28 | 68 |
| 2020 Febrero | 5 | 0 | 5 |
| 2020 Enero | 4 | 0 | 4 |
| 2019 Diciembre | 8 | 0 | 8 |
| 2019 Noviembre | 4 | 0 | 4 |
| 2019 Septiembre | 12 | 0 | 12 |
| 2019 Agosto | 4 | 0 | 4 |
| 2019 Julio | 4 | 0 | 4 |
| 2019 Junio | 6 | 0 | 6 |
| 2019 Mayo | 2 | 0 | 2 |
| 2019 Abril | 4 | 0 | 4 |
| 2019 Marzo | 4 | 0 | 4 |
| 2019 Enero | 2 | 0 | 2 |
| 2018 Diciembre | 2 | 0 | 2 |
| 2018 Noviembre | 1 | 0 | 1 |
| 2018 Octubre | 4 | 0 | 4 |
| 2018 Septiembre | 3 | 0 | 3 |
| 2018 Febrero | 39 | 7 | 46 |
| 2018 Enero | 51 | 8 | 59 |
| 2017 Diciembre | 41 | 9 | 50 |
| 2017 Noviembre | 58 | 6 | 64 |
| 2017 Octubre | 132 | 33 | 165 |
| 2017 Septiembre | 4 | 10 | 14 |
| 2017 Agosto | 4 | 9 | 13 |
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