Case and Research Letters
Dermoscopic Features of Cutaneous Rosai-Dorfman Disease
Características dermatoscópicas de la enfermedad de Rosai-Dorfman cutánea
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Avilés-Izquierdo, V. Parra Blanco, F. Alfageme Roldán" "autores" => array:3 [ 0 => array:4 [ "nombre" => "J.A." "apellidos" => "Avilés-Izquierdo" "email" => array:1 [ 0 => "jaavilesizquierdo@gmail.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" => "V." "apellidos" => "Parra Blanco" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "F." "apellidos" => "Alfageme Roldán" "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 General Universitario Gregorio Marañón, Madrid, Spain" "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Hospital General Universitario Gregorio Marañón’, 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" => "Características dermatoscópicas de la enfermedad de Rosai-Dorfman cutánea" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 710 "Ancho" => 951 "Tamanyo" => 88216 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Dermoscopic image with yellow homogeneous areas surrounded by large telangiectatic vessels on a red-orange background (Fig. 2).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Rosai-Dorfman disease is a benign histiocytic proliferative disorder of unknown etiology. The presenting sign is usually asymptomatic massive lymphadenopathy. Exclusively cutaneous cases of Rosai-Dorfman disease are rare. We present a case report of the disease and describe its dermoscopic features.</p><p id="par0010" class="elsevierStylePara elsevierViewall">A 29-year old man with no relevant medical or surgical history was seen for a lesion that had been growing progressively for several years in the left pectoral region. A biopsy report from another hospital indicated diagnosis of atypical xanthogranuloma.</p><p id="par0015" class="elsevierStylePara elsevierViewall">The physical examination showed an infiltrated plaque with a heterogeneous surface and well-defined borders. Its longest diameter was 4.5<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>4<span class="elsevierStyleHsp" style=""></span>cm and it had an erythematous-orange appearance, a central whitish area, and multiple yellowish structures at the edges (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">The dermoscopic image showed a milky-red area in the center of the lesion, whereas the outer part had multiple yellowish homogeneous areas of different sizes with irregular borders, surrounded by large telangiectatic vessels on a red-orange background (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). Given the progressive growth of the lesion and the discomfort it caused the patient, radical excision was performed.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">Histologic examination revealed a poorly-defined dermal proliferation of large histiocytes. extending downwards into the subcutaneous adipose tissue. The histiocytes had large eosinophilic vacuolated cytoplasm and round homogeneous nuclei and some contained intact lymphocytes (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>). In addition to the histiocytes, there was abundant inflammatory infiltrate composed mainly of plasma cells, with lymphocytes, giant multinucleated cells, and xanthomatous histiocytes with small nuclei. A prominent fibrous stroma was seen with a highly vascularized storiform pattern. Immunohistochemical staining was positive for S-100 and CD68 and negative for CD1a. The presence of emperipolesis and the immunohistochemical profile pointed to diagnosis of a cutaneous variant of Rosai-Dorfman disease. No hematologic abnormalities, lymph node involvement, or abnormalities in other organs were observed in the staging study.</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Rosai-Dorfman disease, or massively enlarged lymph nodes with sinus histiocytosis, is a histiocytic proliferation in which approximately 40% of the patients have extranodal involvement.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> The skin is the most frequently affected organ. Purely cutaneous forms of Rosai-Dorfman disease are rare. Skin lesions are nonspecific and may take the form of solitary or multiple lesions of different sizes and morphologies. They can present on any part of the body and the clinical differential diagnosis includes a broad range of conditions including panniculitis, vasculitis, acne vulgaris, suppurative hidradenitis, granuloma annulare, and sarcoidosis, as well as other histiocytoses.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Kong et al.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> proposed a classification based on the morphologic features of 39 lesions. Papulonodular lesions were the most common form (accounting for almost 80%) followed by the infiltrated plaque type (12.5%) and the tumor type (7.7%). The histologic findings in cutaneous lesions are similar to those found in lymph tissue. The main finding is a dense infiltrate of large histiocytes and a large pale cytoplasm with rounded nuclei. The cytoplasm contains intact leukocytes, usually lymphocytes, a phenomenon known as lymphophagocytosis or emperipolesis. Typically, these histiocytes are positive for S-100 and negative for CD1a, and can be either positive or negative for CD68. The immunohistochemical profile is essential for histologic diagnosis, as fibrosis, vascular proliferation, lymphoid clusters, foam cells, and multinucleated Touton giant cells may or may not be present, and so confusion with other histiocytic processes, and with juvenile xanthogranuloma in particular, is possible.The dermoscopic features of Rosai-Dorfman disease have not been widely reported in the literature.<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5,6</span></a> Rodríguez Blanco et al.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> reported a case of Rosai-Dorfman disease on the sole, characterized by cotton-like ovoid structures on an erythematous background in the dermoscopic image. In contrast, the dermoscopic features in our case were similar to those described for juvenile xanthogranuloma, that is, a yellow-orange homogeneous central area and a somewhat more erythematous peripheral area. This is known as the setting sun feature.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> The presence of clouds of pale yellow globules is considered indicative of xanthomatous histiocytes in the superficial dermis.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> The presence of comma vessels,<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> arborizing telangiectasia,<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> and whitish linear projections has also been reported, particularly in advanced cases of juvenile xanthogranuloma. The differential dermoscopic diagnosis should be performed with solitary yellow lesions, such as juvenile xanthogranuloma, organoid nevus or sebaceous nevus, xanthomatous dermatofibroma, and solitary reticulohistiocytoma.<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">9–11</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The lesion we describe was large, morphologically irregular, with dermoscopic features similar to those described for juvenile xanthogranuloma or solitary reticulohistiocytoma, although our lesion had a wider range of coloration, with a milky-red central zone, multiple yellow clouds surrounded by arborizing vessels, and an erythematous-orange peripheral area.</p><p id="par0040" class="elsevierStylePara elsevierViewall">In conclusion, cutaneous Rosai-Dorfman disease is rare and lacks specific clinical characteristics. It should be suspected when an infiltrate of xanthomatous histiocytes is observed, particularly if these histiocytes contain intact lymphocytes. Given that the differential diagnosis of this disease can be difficult, dermoscopy can be useful, particularly when features with a yellow coloration are present, as in the case presented here.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara">Please cite this article as: Avilés-Izquierdo JA, et al. Características dermatoscópicas de la enfermedad de Rosai-Dorfman cutánea. Actas Dermosifiliogr. 2012;103:446-8.</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" => 708 "Ancho" => 951 "Tamanyo" => 112966 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Yellow-orange plaque in the left pectoral 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" => 710 "Ancho" => 951 "Tamanyo" => 88216 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Dermoscopic image with yellow homogeneous areas surrounded by large telangiectatic vessels on a red-orange background (Fig. 2).</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" => 713 "Ancho" => 951 "Tamanyo" => 212014 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Histologic detail showing eosinophilic vacuolated cytoplasm and round homogeneous nuclei. Intact lymphocytes can be observed inside some of these histiocytes (emperipolesis) (hematoxylin-eosin, original magnification x400).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:11 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "J. Rosai" 1 => "R.F. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 15 | 19 | 34 |
| 2024 October | 66 | 39 | 105 |
| 2024 September | 65 | 19 | 84 |
| 2024 August | 81 | 52 | 133 |
| 2024 July | 64 | 34 | 98 |
| 2024 June | 65 | 30 | 95 |
| 2024 May | 55 | 37 | 92 |
| 2024 April | 75 | 33 | 108 |
| 2024 March | 64 | 33 | 97 |
| 2024 February | 65 | 26 | 91 |
| 2024 January | 45 | 39 | 84 |
| 2023 December | 40 | 21 | 61 |
| 2023 November | 57 | 32 | 89 |
| 2023 October | 66 | 29 | 95 |
| 2023 September | 64 | 40 | 104 |
| 2023 August | 42 | 20 | 62 |
| 2023 July | 38 | 45 | 83 |
| 2023 June | 54 | 32 | 86 |
| 2023 May | 46 | 42 | 88 |
| 2023 April | 41 | 28 | 69 |
| 2023 March | 68 | 38 | 106 |
| 2023 February | 64 | 28 | 92 |
| 2023 January | 73 | 33 | 106 |
| 2022 December | 70 | 45 | 115 |
| 2022 November | 46 | 39 | 85 |
| 2022 October | 39 | 37 | 76 |
| 2022 September | 31 | 36 | 67 |
| 2022 August | 34 | 43 | 77 |
| 2022 July | 28 | 42 | 70 |
| 2022 June | 38 | 35 | 73 |
| 2022 May | 43 | 38 | 81 |
| 2022 April | 36 | 49 | 85 |
| 2022 March | 40 | 48 | 88 |
| 2022 February | 21 | 47 | 68 |
| 2022 January | 37 | 41 | 78 |
| 2021 December | 22 | 39 | 61 |
| 2021 November | 41 | 45 | 86 |
| 2021 October | 41 | 42 | 83 |
| 2021 September | 41 | 41 | 82 |
| 2021 August | 27 | 21 | 48 |
| 2021 July | 28 | 38 | 66 |
| 2021 June | 26 | 30 | 56 |
| 2021 May | 42 | 47 | 89 |
| 2021 April | 53 | 74 | 127 |
| 2021 March | 49 | 41 | 90 |
| 2021 February | 37 | 29 | 66 |
| 2021 January | 26 | 26 | 52 |
| 2020 December | 17 | 12 | 29 |
| 2020 November | 20 | 28 | 48 |
| 2020 October | 27 | 16 | 43 |
| 2020 September | 38 | 13 | 51 |
| 2020 August | 20 | 22 | 42 |
| 2020 July | 32 | 24 | 56 |
| 2020 June | 32 | 27 | 59 |
| 2020 May | 33 | 23 | 56 |
| 2020 April | 35 | 19 | 54 |
| 2020 March | 30 | 20 | 50 |
| 2020 February | 10 | 3 | 13 |
| 2020 January | 0 | 4 | 4 |
| 2019 December | 6 | 4 | 10 |
| 2019 November | 6 | 0 | 6 |
| 2019 October | 0 | 1 | 1 |
| 2019 September | 8 | 2 | 10 |
| 2019 August | 4 | 0 | 4 |
| 2019 July | 4 | 8 | 12 |
| 2019 June | 4 | 14 | 18 |
| 2019 May | 7 | 42 | 49 |
| 2019 April | 2 | 9 | 11 |
| 2019 March | 4 | 3 | 7 |
| 2019 February | 2 | 3 | 5 |
| 2019 January | 1 | 0 | 1 |
| 2018 December | 2 | 0 | 2 |
| 2018 November | 2 | 0 | 2 |
| 2018 October | 3 | 0 | 3 |
| 2018 September | 2 | 0 | 2 |
| 2018 March | 1 | 0 | 1 |
| 2018 February | 100 | 3 | 103 |
| 2018 January | 56 | 8 | 64 |
| 2017 December | 97 | 12 | 109 |
| 2017 November | 33 | 3 | 36 |
| 2017 October | 54 | 9 | 63 |
| 2017 September | 52 | 10 | 62 |
| 2017 August | 68 | 10 | 78 |
| 2017 July | 41 | 6 | 47 |
| 2017 June | 69 | 12 | 81 |
| 2017 May | 71 | 7 | 78 |
| 2017 April | 55 | 23 | 78 |
| 2017 March | 69 | 16 | 85 |
| 2017 February | 45 | 10 | 55 |
| 2017 January | 27 | 10 | 37 |
| 2016 December | 48 | 6 | 54 |
| 2016 November | 70 | 10 | 80 |
| 2016 October | 57 | 7 | 64 |
| 2016 September | 95 | 14 | 109 |
| 2016 August | 68 | 11 | 79 |
| 2016 July | 45 | 10 | 55 |
| 2016 June | 14 | 25 | 39 |
| 2016 May | 9 | 11 | 20 |
| 2016 April | 4 | 5 | 9 |
| 2016 March | 8 | 2 | 10 |
| 2016 February | 7 | 1 | 8 |
| 2016 January | 4 | 2 | 6 |
| 2015 December | 7 | 0 | 7 |
| 2015 November | 27 | 2 | 29 |
| 2015 October | 21 | 4 | 25 |
| 2015 September | 9 | 1 | 10 |
| 2015 August | 10 | 3 | 13 |
| 2015 July | 82 | 11 | 93 |
| 2015 June | 73 | 8 | 81 |
| 2015 May | 124 | 11 | 135 |
| 2015 April | 92 | 12 | 104 |
| 2015 March | 78 | 5 | 83 |
| 2015 February | 81 | 12 | 93 |
| 2015 January | 57 | 22 | 79 |
| 2014 December | 67 | 11 | 78 |
| 2014 November | 62 | 10 | 72 |
| 2014 October | 74 | 20 | 94 |
| 2014 September | 59 | 12 | 71 |
| 2014 August | 67 | 12 | 79 |
| 2014 July | 68 | 14 | 82 |
| 2014 June | 79 | 12 | 91 |
| 2014 May | 40 | 5 | 45 |
| 2014 April | 40 | 3 | 43 |
| 2014 March | 45 | 9 | 54 |
| 2014 February | 35 | 15 | 50 |
| 2014 January | 40 | 7 | 47 |
| 2013 December | 33 | 11 | 44 |
| 2013 November | 39 | 13 | 52 |
| 2013 October | 23 | 9 | 32 |
| 2013 September | 26 | 13 | 39 |
| 2013 August | 29 | 19 | 48 |
| 2013 July | 19 | 32 | 51 |
| 2013 June | 18 | 28 | 46 |
| 2013 May | 14 | 18 | 32 |
| 2013 April | 10 | 29 | 39 |
| 2013 March | 17 | 22 | 39 |
| 2013 February | 34 | 6 | 40 |
| 2013 January | 43 | 7 | 50 |
| 2012 December | 21 | 4 | 25 |
| 2012 November | 1 | 1 | 2 |
| 2012 October | 2 | 3 | 5 |
| 2012 August | 2 | 1 | 3 |
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