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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Rosai-Dorfman disease is a benign histiocytic proliferative disorder of unknown etiology&#46; The presenting sign is usually asymptomatic massive lymphadenopathy&#46; Exclusively cutaneous cases of Rosai-Dorfman disease are rare&#46; We present a case report of the disease and describe its dermoscopic features&#46;</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&#46; A biopsy report from another hospital indicated diagnosis of atypical xanthogranuloma&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">The physical examination showed an infiltrated plaque with a heterogeneous surface and well-defined borders&#46; Its longest diameter was 4&#46;5<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>4<span class="elsevierStyleHsp" style=""></span>cm and it had an erythematous-orange appearance&#44; a central whitish area&#44; and multiple yellowish structures at the edges &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46;</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&#44; whereas the outer part had multiple yellowish homogeneous areas of different sizes with irregular borders&#44; surrounded by large telangiectatic vessels on a red-orange background &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Given the progressive growth of the lesion and the discomfort it caused the patient&#44; radical excision was performed&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">Histologic examination revealed a poorly-defined dermal proliferation of large histiocytes&#46; extending downwards into the subcutaneous adipose tissue&#46; The histiocytes had large eosinophilic vacuolated cytoplasm and round homogeneous nuclei and some contained intact lymphocytes &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; In addition to the histiocytes&#44; there was abundant inflammatory infiltrate composed mainly of plasma cells&#44; with lymphocytes&#44; giant multinucleated cells&#44; and xanthomatous histiocytes with small nuclei&#46; A prominent fibrous stroma was seen with a highly vascularized storiform pattern&#46; Immunohistochemical staining was positive for S-100 and CD68 and negative for CD1a&#46; The presence of emperipolesis and the immunohistochemical profile pointed to diagnosis of a cutaneous variant of Rosai-Dorfman disease&#46; No hematologic abnormalities&#44; lymph node involvement&#44; or abnormalities in other organs were observed in the staging study&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Rosai-Dorfman disease&#44; or massively enlarged lymph nodes with sinus histiocytosis&#44; is a histiocytic proliferation in which approximately 40&#37; of the patients have extranodal involvement&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2</span></a> The skin is the most frequently affected organ&#46; Purely cutaneous forms of Rosai-Dorfman disease are rare&#46; Skin lesions are nonspecific and may take the form of solitary or multiple lesions of different sizes and morphologies&#46; They can present on any part of the body and the clinical differential diagnosis includes a broad range of conditions including panniculitis&#44; vasculitis&#44; acne vulgaris&#44; suppurative hidradenitis&#44; granuloma annulare&#44; and sarcoidosis&#44; as well as other histiocytoses&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Kong et al&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> proposed a classification based on the morphologic features of 39 lesions&#46; Papulonodular lesions were the most common form &#40;accounting for almost 80&#37;&#41; followed by the infiltrated plaque type &#40;12&#46;5&#37;&#41; and the tumor type &#40;7&#46;7&#37;&#41;&#46; The histologic findings in cutaneous lesions are similar to those found in lymph tissue&#46; The main finding is a dense infiltrate of large histiocytes and a large pale cytoplasm with rounded nuclei&#46; The cytoplasm contains intact leukocytes&#44; usually lymphocytes&#44; a phenomenon known as lymphophagocytosis or emperipolesis&#46; Typically&#44; these histiocytes are positive for S-100 and negative for CD1a&#44; and can be either positive or negative for CD68&#46; The immunohistochemical profile is essential for histologic diagnosis&#44; as fibrosis&#44; vascular proliferation&#44; lymphoid clusters&#44; foam cells&#44; and multinucleated Touton giant cells may or may not be present&#44; and so confusion with other histiocytic processes&#44; and with juvenile xanthogranuloma in particular&#44; is possible&#46;The dermoscopic features of Rosai-Dorfman disease have not been widely reported in the literature&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;6</span></a> Rodr&#237;guez Blanco et al&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> reported a case of Rosai-Dorfman disease on the sole&#44; characterized by cotton-like ovoid structures on an erythematous background in the dermoscopic image&#46; In contrast&#44; the dermoscopic features in our case were similar to those described for juvenile xanthogranuloma&#44; that is&#44; a yellow-orange homogeneous central area and a somewhat more erythematous peripheral area&#46; This is known as the setting sun feature&#46;<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&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> The presence of comma vessels&#44;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> arborizing telangiectasia&#44;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> and whitish linear projections has also been reported&#44; particularly in advanced cases of juvenile xanthogranuloma&#46; The differential dermoscopic diagnosis should be performed with solitary yellow lesions&#44; such as juvenile xanthogranuloma&#44; organoid nevus or sebaceous nevus&#44; xanthomatous dermatofibroma&#44; and solitary reticulohistiocytoma&#46;<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">9&#8211;11</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The lesion we describe was large&#44; morphologically irregular&#44; with dermoscopic features similar to those described for juvenile xanthogranuloma or solitary reticulohistiocytoma&#44; although our lesion had a wider range of coloration&#44; with a milky-red central zone&#44; multiple yellow clouds surrounded by arborizing vessels&#44; and an erythematous-orange peripheral area&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">In conclusion&#44; cutaneous Rosai-Dorfman disease is rare and lacks specific clinical characteristics&#46; It should be suspected when an infiltrate of xanthomatous histiocytes is observed&#44; particularly if these histiocytes contain intact lymphocytes&#46; Given that the differential diagnosis of this disease can be difficult&#44; dermoscopy can be useful&#44; particularly when features with a yellow coloration are present&#44; as in the case presented here&#46;</p></span>"
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Case and Research Letters
Dermoscopic Features of Cutaneous Rosai-Dorfman Disease
Características dermatoscópicas de la enfermedad de Rosai-Dorfman cutánea
J.A. Avilés-Izquierdoa,
Autor para correspondencia
jaavilesizquierdo@gmail.com

Corresponding author.
, V. Parra Blancob, F. Alfageme Roldána
a Servicio de Dermatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain
b Servicio de Anatomía Patológica, Hospital General Universitario Gregorio Marañón’, Madrid, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Rosai-Dorfman disease is a benign histiocytic proliferative disorder of unknown etiology&#46; The presenting sign is usually asymptomatic massive lymphadenopathy&#46; Exclusively cutaneous cases of Rosai-Dorfman disease are rare&#46; We present a case report of the disease and describe its dermoscopic features&#46;</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&#46; A biopsy report from another hospital indicated diagnosis of atypical xanthogranuloma&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">The physical examination showed an infiltrated plaque with a heterogeneous surface and well-defined borders&#46; Its longest diameter was 4&#46;5<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>4<span class="elsevierStyleHsp" style=""></span>cm and it had an erythematous-orange appearance&#44; a central whitish area&#44; and multiple yellowish structures at the edges &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46;</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&#44; whereas the outer part had multiple yellowish homogeneous areas of different sizes with irregular borders&#44; surrounded by large telangiectatic vessels on a red-orange background &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Given the progressive growth of the lesion and the discomfort it caused the patient&#44; radical excision was performed&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">Histologic examination revealed a poorly-defined dermal proliferation of large histiocytes&#46; extending downwards into the subcutaneous adipose tissue&#46; The histiocytes had large eosinophilic vacuolated cytoplasm and round homogeneous nuclei and some contained intact lymphocytes &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; In addition to the histiocytes&#44; there was abundant inflammatory infiltrate composed mainly of plasma cells&#44; with lymphocytes&#44; giant multinucleated cells&#44; and xanthomatous histiocytes with small nuclei&#46; A prominent fibrous stroma was seen with a highly vascularized storiform pattern&#46; Immunohistochemical staining was positive for S-100 and CD68 and negative for CD1a&#46; The presence of emperipolesis and the immunohistochemical profile pointed to diagnosis of a cutaneous variant of Rosai-Dorfman disease&#46; No hematologic abnormalities&#44; lymph node involvement&#44; or abnormalities in other organs were observed in the staging study&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Rosai-Dorfman disease&#44; or massively enlarged lymph nodes with sinus histiocytosis&#44; is a histiocytic proliferation in which approximately 40&#37; of the patients have extranodal involvement&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2</span></a> The skin is the most frequently affected organ&#46; Purely cutaneous forms of Rosai-Dorfman disease are rare&#46; Skin lesions are nonspecific and may take the form of solitary or multiple lesions of different sizes and morphologies&#46; They can present on any part of the body and the clinical differential diagnosis includes a broad range of conditions including panniculitis&#44; vasculitis&#44; acne vulgaris&#44; suppurative hidradenitis&#44; granuloma annulare&#44; and sarcoidosis&#44; as well as other histiocytoses&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Kong et al&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> proposed a classification based on the morphologic features of 39 lesions&#46; Papulonodular lesions were the most common form &#40;accounting for almost 80&#37;&#41; followed by the infiltrated plaque type &#40;12&#46;5&#37;&#41; and the tumor type &#40;7&#46;7&#37;&#41;&#46; The histologic findings in cutaneous lesions are similar to those found in lymph tissue&#46; The main finding is a dense infiltrate of large histiocytes and a large pale cytoplasm with rounded nuclei&#46; The cytoplasm contains intact leukocytes&#44; usually lymphocytes&#44; a phenomenon known as lymphophagocytosis or emperipolesis&#46; Typically&#44; these histiocytes are positive for S-100 and negative for CD1a&#44; and can be either positive or negative for CD68&#46; The immunohistochemical profile is essential for histologic diagnosis&#44; as fibrosis&#44; vascular proliferation&#44; lymphoid clusters&#44; foam cells&#44; and multinucleated Touton giant cells may or may not be present&#44; and so confusion with other histiocytic processes&#44; and with juvenile xanthogranuloma in particular&#44; is possible&#46;The dermoscopic features of Rosai-Dorfman disease have not been widely reported in the literature&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;6</span></a> Rodr&#237;guez Blanco et al&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> reported a case of Rosai-Dorfman disease on the sole&#44; characterized by cotton-like ovoid structures on an erythematous background in the dermoscopic image&#46; In contrast&#44; the dermoscopic features in our case were similar to those described for juvenile xanthogranuloma&#44; that is&#44; a yellow-orange homogeneous central area and a somewhat more erythematous peripheral area&#46; This is known as the setting sun feature&#46;<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&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> The presence of comma vessels&#44;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> arborizing telangiectasia&#44;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> and whitish linear projections has also been reported&#44; particularly in advanced cases of juvenile xanthogranuloma&#46; The differential dermoscopic diagnosis should be performed with solitary yellow lesions&#44; such as juvenile xanthogranuloma&#44; organoid nevus or sebaceous nevus&#44; xanthomatous dermatofibroma&#44; and solitary reticulohistiocytoma&#46;<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">9&#8211;11</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The lesion we describe was large&#44; morphologically irregular&#44; with dermoscopic features similar to those described for juvenile xanthogranuloma or solitary reticulohistiocytoma&#44; although our lesion had a wider range of coloration&#44; with a milky-red central zone&#44; multiple yellow clouds surrounded by arborizing vessels&#44; and an erythematous-orange peripheral area&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">In conclusion&#44; cutaneous Rosai-Dorfman disease is rare and lacks specific clinical characteristics&#46; It should be suspected when an infiltrate of xanthomatous histiocytes is observed&#44; particularly if these histiocytes contain intact lymphocytes&#46; Given that the differential diagnosis of this disease can be difficult&#44; dermoscopy can be useful&#44; particularly when features with a yellow coloration are present&#44; as in the case presented here&#46;</p></span>"
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ISSN: 15782190
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