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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Medical History</span><p id="par0005" class="elsevierStylePara elsevierViewall">A 48-year-old woman with no previous history of interest was assessed in the dermatology department for a lesion on the mons pubis that had first appeared several months earlier&#46; The lesion had not responded to intralesional corticosteroids&#46; The patient reported no scarring or previous folliculitis&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">Physical examination revealed a painless indurated multinodular plaque with a smooth surface and well-defined borders measuring 4<span class="elsevierStyleHsp" style=""></span>cm in diameter on the mons pubis &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; The patient also had 2 erythematous papular lesions measuring less than 1<span class="elsevierStyleHsp" style=""></span>cm on both sides of the upper chest&#46; She had no systemic symptoms or enlarged lymph nodes&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Biopsy revealed a normal epidermis and a dense polymorphous infiltrate in the middle and deep dermis&#44; as well as increased collagen fibrils and vascularization &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; There was evidence of emperipolesis&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Immunohistochemistry revealed positive findings for CD68&#44; S100 &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#44; and cyclin D1 and negative findings for CD1a&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Additional Tests</span><p id="par0025" class="elsevierStylePara elsevierViewall">An extension study was performed with computed tomography and a full laboratory workup&#44; neither of which revealed abnormalities&#46; Similarly&#44; serology testing was negative for Epstein-Barr virus&#44; cytomegalovirus&#44; human herpesvirus &#40;HHV&#41; 6&#44; HHV8&#44; HIV&#44; and syphilis&#44; and skin cultures were negative for <span class="elsevierStyleItalic">Mycobacterium</span> and <span class="elsevierStyleItalic">Treponema</span> species&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">What is your diagnosis&#63;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Diagnosis</span><p id="par0035" class="elsevierStylePara elsevierViewall">Cutaneous Rosai-Dorfman disease&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical Course and Treatment</span><p id="par0040" class="elsevierStylePara elsevierViewall">The lesions were surgically removed&#46; A new lesion appeared on the patient&#39;s upper chest after 4 months of follow-up&#46; This was removed&#44; and the same histology findings were reported&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Comment</span><p id="par0045" class="elsevierStylePara elsevierViewall">Rosai-Dorfman disease is a proliferative non-Langerhans cell histiocytic disorder that generally progresses with the presence of massive painless bilateral enlarged lymph nodes&#44; systemic symptoms&#44; increased acute phase reactant values&#44; and hypergammaglobulinemia&#46; Extranodal manifestations are observed in up to 40&#37; of patients&#44; with the skin affected in approximately 10&#37;&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">One form of Rosai-Dorfman disease only affects the skin&#46; Mean age at onset is higher than that of the classic form &#40;43&#46;5 vs&#46; 20&#46;6 years&#41;&#44; and the disease predominantly affects women&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The most common clinical presentation is the papulonodular form&#44; although a wide variety of lesions have been reported &#40;indurated plaques&#44; tumors&#44; acneiform lesions&#44; xanthogranulomatous lesions&#44; and ulcerations&#41;&#46; Consequently&#44; the differential diagnosis is very varied and includes histiocytic&#44; inflammatory&#44; and tumor-like proliferations &#40;e&#46;g&#46;&#44; cutaneous lymphoma&#44; Kaposi sarcoma&#44; dermatofibrosarcoma protuberans&#41;&#44; as well as skin infections caused by mycobacteria&#46; The disease mainly affects the thorax&#44; and there is frequently more than 1 lesion at diagnosis&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">Rosai-Dorfman disease has been associated with viral infections&#44; especially those caused by herpesvirus&#44; and mutations in MAP2K1&#47;ERK&#44; proteins that form part of one of the pathways that regulate cell survival and proliferation&#46; An association has also been established with autoimmune diseases&#44; such as systemic lupus erythematosus and juvenile idiopathic arthritis&#44; as well as with lymphoid and myeloid neoplasms&#46; A similar form has been described with mutations in <span class="elsevierStyleItalic">SLC29A3</span> in the germline&#46; This gene has been involved in H syndrome and in pigmented hypertrichosis with insulin-dependent diabetes mellitus&#46; Lastly&#44; high levels of IgG4 have been detected&#44; especially in the classic form with visceral involvement&#44; thus opening the debate on whether this condition should be included within IgG4-related diseases&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Diagnosis is based on histopathology of the lesion and on a set of additional tests to rule disease at other levels&#46; These include imaging tests&#44; mainly computed tomography&#44; and a laboratory workup comprising a complete blood count&#44; biochemistry&#44; kidney and liver function&#44; antinuclear antibodies&#44; complement&#44; proteinogram&#44; and serology testing for Epstein-Barr virus&#44; cytomegalovirus&#44; HHV6&#44; HHV8&#44; HIV&#44; and syphilis&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">Biopsy reveals a dense histiocytic infiltrate in the dermis with lymphocytes&#44; plasma cells&#44; and disperse neutrophils&#46; The presence of emperipolesis is a key diagnostic finding&#46; Immunohistochemistry shows the origin of the cell strain that makes up the lesion and is positive for CD14&#44; S100&#44; CD68&#44; and CD163 &#40;histocyte markers&#41; and negative for CD1a&#44; langerin &#40;Langerhans cell markers&#41;&#44; and factor XIIIa&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">The lesion can be treated with surgery&#44;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> as well as with oral and intralesional corticosteroids&#44; methotrexate&#44;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a> topical imiquimod&#44;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a> and cryotherapy&#46; Isolated cases treated using these approaches have been reported&#46; Prognosis is generally good&#44; potentially with spontaneous remission of the lesions in up to 20&#37; of cases&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a></p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicts of Interest</span><p id="par0080" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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Casos para el diagnóstico
Recent-Onset Indurated Plaque on the Mons Pubis
Una placa indurada en el pubis de reciente aparición
C. Fernández Romeroa,
Autor para correspondencia
nefercrisss@gmail.com

Corresponding author.
, E. Gimeno Carpioa, V.C. Puglia Santosb
a Servicio de Dermatología, Hospital Arnau de Vilanova, Valencia, Spain
b Servicio de Anatomía Patológica, Hospital Arnau de Vilanova, Valencia, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Medical History</span><p id="par0005" class="elsevierStylePara elsevierViewall">A 48-year-old woman with no previous history of interest was assessed in the dermatology department for a lesion on the mons pubis that had first appeared several months earlier&#46; The lesion had not responded to intralesional corticosteroids&#46; The patient reported no scarring or previous folliculitis&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">Physical examination revealed a painless indurated multinodular plaque with a smooth surface and well-defined borders measuring 4<span class="elsevierStyleHsp" style=""></span>cm in diameter on the mons pubis &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; The patient also had 2 erythematous papular lesions measuring less than 1<span class="elsevierStyleHsp" style=""></span>cm on both sides of the upper chest&#46; She had no systemic symptoms or enlarged lymph nodes&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Biopsy revealed a normal epidermis and a dense polymorphous infiltrate in the middle and deep dermis&#44; as well as increased collagen fibrils and vascularization &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; There was evidence of emperipolesis&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Immunohistochemistry revealed positive findings for CD68&#44; S100 &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#44; and cyclin D1 and negative findings for CD1a&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Additional Tests</span><p id="par0025" class="elsevierStylePara elsevierViewall">An extension study was performed with computed tomography and a full laboratory workup&#44; neither of which revealed abnormalities&#46; Similarly&#44; serology testing was negative for Epstein-Barr virus&#44; cytomegalovirus&#44; human herpesvirus &#40;HHV&#41; 6&#44; HHV8&#44; HIV&#44; and syphilis&#44; and skin cultures were negative for <span class="elsevierStyleItalic">Mycobacterium</span> and <span class="elsevierStyleItalic">Treponema</span> species&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">What is your diagnosis&#63;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Diagnosis</span><p id="par0035" class="elsevierStylePara elsevierViewall">Cutaneous Rosai-Dorfman disease&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical Course and Treatment</span><p id="par0040" class="elsevierStylePara elsevierViewall">The lesions were surgically removed&#46; A new lesion appeared on the patient&#39;s upper chest after 4 months of follow-up&#46; This was removed&#44; and the same histology findings were reported&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Comment</span><p id="par0045" class="elsevierStylePara elsevierViewall">Rosai-Dorfman disease is a proliferative non-Langerhans cell histiocytic disorder that generally progresses with the presence of massive painless bilateral enlarged lymph nodes&#44; systemic symptoms&#44; increased acute phase reactant values&#44; and hypergammaglobulinemia&#46; Extranodal manifestations are observed in up to 40&#37; of patients&#44; with the skin affected in approximately 10&#37;&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">One form of Rosai-Dorfman disease only affects the skin&#46; Mean age at onset is higher than that of the classic form &#40;43&#46;5 vs&#46; 20&#46;6 years&#41;&#44; and the disease predominantly affects women&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The most common clinical presentation is the papulonodular form&#44; although a wide variety of lesions have been reported &#40;indurated plaques&#44; tumors&#44; acneiform lesions&#44; xanthogranulomatous lesions&#44; and ulcerations&#41;&#46; Consequently&#44; the differential diagnosis is very varied and includes histiocytic&#44; inflammatory&#44; and tumor-like proliferations &#40;e&#46;g&#46;&#44; cutaneous lymphoma&#44; Kaposi sarcoma&#44; dermatofibrosarcoma protuberans&#41;&#44; as well as skin infections caused by mycobacteria&#46; The disease mainly affects the thorax&#44; and there is frequently more than 1 lesion at diagnosis&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">Rosai-Dorfman disease has been associated with viral infections&#44; especially those caused by herpesvirus&#44; and mutations in MAP2K1&#47;ERK&#44; proteins that form part of one of the pathways that regulate cell survival and proliferation&#46; An association has also been established with autoimmune diseases&#44; such as systemic lupus erythematosus and juvenile idiopathic arthritis&#44; as well as with lymphoid and myeloid neoplasms&#46; A similar form has been described with mutations in <span class="elsevierStyleItalic">SLC29A3</span> in the germline&#46; This gene has been involved in H syndrome and in pigmented hypertrichosis with insulin-dependent diabetes mellitus&#46; Lastly&#44; high levels of IgG4 have been detected&#44; especially in the classic form with visceral involvement&#44; thus opening the debate on whether this condition should be included within IgG4-related diseases&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Diagnosis is based on histopathology of the lesion and on a set of additional tests to rule disease at other levels&#46; These include imaging tests&#44; mainly computed tomography&#44; and a laboratory workup comprising a complete blood count&#44; biochemistry&#44; kidney and liver function&#44; antinuclear antibodies&#44; complement&#44; proteinogram&#44; and serology testing for Epstein-Barr virus&#44; cytomegalovirus&#44; HHV6&#44; HHV8&#44; HIV&#44; and syphilis&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">Biopsy reveals a dense histiocytic infiltrate in the dermis with lymphocytes&#44; plasma cells&#44; and disperse neutrophils&#46; The presence of emperipolesis is a key diagnostic finding&#46; Immunohistochemistry shows the origin of the cell strain that makes up the lesion and is positive for CD14&#44; S100&#44; CD68&#44; and CD163 &#40;histocyte markers&#41; and negative for CD1a&#44; langerin &#40;Langerhans cell markers&#41;&#44; and factor XIIIa&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">The lesion can be treated with surgery&#44;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> as well as with oral and intralesional corticosteroids&#44; methotrexate&#44;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a> topical imiquimod&#44;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a> and cryotherapy&#46; Isolated cases treated using these approaches have been reported&#46; Prognosis is generally good&#44; potentially with spontaneous remission of the lesions in up to 20&#37; of cases&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a></p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicts of Interest</span><p id="par0080" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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