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
Corresponding author
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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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 8 | 17 | 25 |
| 2024 October | 84 | 40 | 124 |
| 2024 September | 97 | 33 | 130 |
| 2024 August | 127 | 85 | 212 |
| 2024 July | 91 | 49 | 140 |
| 2024 June | 79 | 60 | 139 |
| 2024 May | 60 | 47 | 107 |
| 2024 April | 77 | 27 | 104 |
| 2024 March | 68 | 25 | 93 |
| 2024 February | 57 | 35 | 92 |
| 2024 January | 52 | 42 | 94 |
| 2023 December | 58 | 14 | 72 |
| 2023 November | 88 | 27 | 115 |
| 2023 October | 68 | 24 | 92 |
| 2023 September | 58 | 26 | 84 |
| 2023 August | 45 | 12 | 57 |
| 2023 July | 66 | 32 | 98 |
| 2023 June | 71 | 25 | 96 |
| 2023 May | 72 | 22 | 94 |
| 2023 April | 78 | 19 | 97 |
| 2023 March | 64 | 18 | 82 |
| 2023 February | 62 | 16 | 78 |
| 2023 January | 59 | 30 | 89 |
| 2022 December | 82 | 35 | 117 |
| 2022 November | 30 | 28 | 58 |
| 2022 October | 34 | 20 | 54 |
| 2022 September | 41 | 45 | 86 |
| 2022 August | 40 | 26 | 66 |
| 2022 July | 33 | 42 | 75 |
| 2022 June | 27 | 22 | 49 |
| 2022 May | 60 | 36 | 96 |
| 2022 April | 66 | 30 | 96 |
| 2022 March | 57 | 53 | 110 |
| 2022 February | 42 | 27 | 69 |
| 2022 January | 89 | 33 | 122 |
| 2021 December | 63 | 41 | 104 |
| 2021 November | 65 | 37 | 102 |
| 2021 October | 56 | 55 | 111 |
| 2021 September | 53 | 27 | 80 |
| 2021 August | 35 | 31 | 66 |
| 2021 July | 53 | 30 | 83 |
| 2021 June | 41 | 23 | 64 |
| 2021 May | 41 | 41 | 82 |
| 2021 April | 101 | 79 | 180 |
| 2021 March | 78 | 38 | 116 |
| 2021 February | 72 | 28 | 100 |
| 2021 January | 57 | 18 | 75 |
| 2020 December | 49 | 17 | 66 |
| 2020 November | 42 | 14 | 56 |
| 2020 October | 29 | 14 | 43 |
| 2020 September | 52 | 24 | 76 |
| 2020 August | 27 | 23 | 50 |
| 2020 July | 37 | 18 | 55 |
| 2020 June | 40 | 31 | 71 |
| 2020 May | 28 | 25 | 53 |
| 2020 April | 30 | 15 | 45 |
| 2020 March | 40 | 28 | 68 |
| 2020 February | 5 | 0 | 5 |
| 2020 January | 4 | 0 | 4 |
| 2019 December | 8 | 0 | 8 |
| 2019 November | 4 | 0 | 4 |
| 2019 September | 12 | 0 | 12 |
| 2019 August | 4 | 0 | 4 |
| 2019 July | 4 | 0 | 4 |
| 2019 June | 6 | 0 | 6 |
| 2019 May | 2 | 0 | 2 |
| 2019 April | 4 | 0 | 4 |
| 2019 March | 4 | 0 | 4 |
| 2019 January | 2 | 0 | 2 |
| 2018 December | 2 | 0 | 2 |
| 2018 November | 1 | 0 | 1 |
| 2018 October | 4 | 0 | 4 |
| 2018 September | 3 | 0 | 3 |
| 2018 February | 39 | 7 | 46 |
| 2018 January | 51 | 8 | 59 |
| 2017 December | 41 | 9 | 50 |
| 2017 November | 58 | 6 | 64 |
| 2017 October | 132 | 33 | 165 |
| 2017 September | 4 | 10 | 14 |
| 2017 August | 4 | 9 | 13 |
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