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Physical examination uncovered a large painless left submandibular mass (5<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>3<span class="elsevierStyleHsp" style=""></span>cm), with multiple smaller ones bilaterally. Computed tomography showed multiple cervical and mediastinal adenopathies, more exuberantly on the left lateral lymphatic chain (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). Histology revealed macrophages in a background with eosinophils and emperipolesis (phagocytosis of intact lymphocytes and other immune cells by histiocytes) suggesting Rosai–Dorfman Disease (RDD) (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Also known as sinus histiocytosis with massive lymphadenopathy, RDD is an extremely rare disorder of unknown origin, whose diagnosis is histological, characterized by emperipolesis (phagocytosis of intact lymphocytes and other immune cells by histiocytes).</p><p id="par0020" class="elsevierStylePara elsevierViewall">Predominantly appears in the first two decades, with male-to-female ratio of 5:1.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Clinically causes massive painless lymphadenopathies (most cervical), fever (30%) and extra-nodal involvement (40%).</p><p id="par0030" class="elsevierStylePara elsevierViewall">Differential diagnosis includes reactive lymph node hyperplasia, tuberculosis or lymphoma, among others.</p><p id="par0035" class="elsevierStylePara elsevierViewall">Even though a benign self-limiting disease, treatment may be necessary, although still controversial.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:2 [ 0 => array:6 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 853 "Ancho" => 900 "Tamanyo" => 47710 ] ] ] 1 => array:6 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 675 "Ancho" => 900 "Tamanyo" => 214806 ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/23870206/0000015300000009/v1_201911071124/S2387020619304279/v1_201911071124/en/main.assets" "Apartado" => array:4 [ "identificador" => "51820" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Images in medicine" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23870206/0000015300000009/v1_201911071124/S2387020619304279/v1_201911071124/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020619304279?idApp=UINPBA00004N" ]
Journal Information
Image in medicine
Rosai–Dorfman Disease: A rare cause of massive lymphadenopathy
Enfermedad de Rosai-Dorfman: una causa rara de linfadenopatía masiva
Marta Custódio
, Isa Silva, Anabela Salgueiro Marques
Corresponding author
Cascais Hospital, Portugal