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lymphocytes&#44; and cells with abundant cytoplasm and reniform nuclei &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#44; which were positive for S-100 and CD1a &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">The following tests were carried out&#58; complete blood count&#44; biochemistry profile&#44; skeletal survey&#44; chest radiograph&#44; abdominal ultrasound&#44; and full physical examination&#46; All results were normal&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">What Is Your Diagnosis&#63;</span></p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Unifocal Langerhans cell histiocytosis &#40;LCH&#41; of the skin &#40;cutaneous eosinophilic granuloma&#41;&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">The lesion was surgically excised and the patient has been followed up for 18 months with no local or systemic recurrence&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0040" class="elsevierStylePara elsevierViewall">LCH is characterized by an infiltrate of cells with Langerhans cell phenotype and can affect various organs&#44; including the skin&#44; mucosas&#44; lymph nodes&#44; liver&#44; spleen&#44; bone&#44; digestive tract&#44; thymus&#44; and pituitary gland&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> According to some studies&#44; skin lesions are present in up to 50&#37; of patients in the initial stages of LCH and can therefore be decisive in establishing a diagnosis&#59; however&#44; because clinical presentation varies so widely&#44; LCH is often not suspected&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">LCH has traditionally been classified as follows&#58; eosinophilic granuloma &#40;single-site disease&#41;&#44; Hand-Sch&#252;ller-Christian disease &#40;chronic&#44; progressive&#44; multifocal disease&#41;&#44; and Letterer-Siwe disease &#40;acute&#44; diffuse disease with a poor prognosis&#41;&#46; In recent years&#44; however&#44; the Histiocyte Society has recommended classifying LCH by site and extent&#44; as follows&#58; type 1&#44; unifocal&#59; type 2&#44; multifocal without involvement of the liver&#44; spleen&#44; lungs&#44; or bone marrow&#59; type 3&#44; multifocal with involvement of the liver&#44; spleen&#44; lungs&#44; and&#47;or bone marrow&#44; or age less than 2 years&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">The typical&#44; classical LCH skin lesions are described as small&#44; purpuric&#44; infiltrated papules with a crusted or scaly surface on the scalp&#44; face&#44; flexures&#44; genital region&#44; and trunk&#46; However&#44; it should be noted that a wide variety of skin lesions of various morphologies can be found in LCH&#44;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> including the following&#58; blisters&#59; vesicles&#59; pustules&#59; single or multiple papules that can be skin-colored&#44; pink&#44; xanthomatous&#44; or purpuric&#59; tumors&#59; nail dystrophy&#59;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> and mucosal ulcerations&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> As a result&#44; the differential diagnosis is very extensive and includes Darier disease&#44; cutaneous candidiasis&#44; non-Langerhans cell histiocytosis&#44; intertrigo&#44; vasculitis&#44; urticaria pigmentosa&#44; sarcoidosis&#44; mycosis fungoides&#44; impetigo&#44; folliculitis&#44; herpes simplex&#44; and chickenpox&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">LCH is diagnosed histologically&#46; Skin lesions&#44; because of their accessibility&#44; are often the key to diagnosis&#46; Histologic examination reveals an infiltrate of histiocytoid cells with reniform nuclei that express CD1a and S-100&#46; Intracytoplasmic Birbeck granules are visible with electron microscopy&#46; In the case of our patient&#44; the late presentation of a supposed epidermal nevus at age 8 years prompted us to order the histologic study&#44; which led us to the correct diagnosis&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">Unifocal forms of LCH are usually found on bone&#44; most frequently the cranium and long bones&#46; Unifocal manifestation on the skin&#8212;as in the reported case&#8212;is rare&#46; These localized cases can be treated with nitrogen mustard&#44; psoralen&#8211;UV-A therapy&#44; surgery&#44; intralesional corticosteroids&#44; radiation therapy&#44; or thalidomide&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Prognosis is determined by the number of organs involved and will be worse if the liver&#44; lungs&#44; or bone marrow are affected&#46; Localized cutaneous forms of LCH have a good prognosis and rarely progress to multisystem disease&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a></p></span></span>"
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Case for Diagnosis
Pink Papules Arranged Linearly
Pápulas rosadas de distribución lineal
L. Campos-Muñoza,
Corresponding author
luciacampos78@hotmail.com

Corresponding author.
, R. Ramírez-Morab, E. López-Brana
a Servicio de Dermatología, Hospital Clínico San Carlos, Madrid, Spain
b Servicio de Anatomía Patológica, Hospital Clínico San Carlos, Madrid, 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">An 8-year-old girl with no relevant past history presented with asymptomatic lesions on the right axilla that had remained stable since they first appeared 2 years earlier&#46; The patient was asymptomatic&#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 several pink and brown papules arranged linearly on the floor of the right axilla &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#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">Histopathologic findings showed a dense dermal infiltrate composed of eosinophils&#44; lymphocytes&#44; and cells with abundant cytoplasm and reniform nuclei &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#44; which were positive for S-100 and CD1a &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">The following tests were carried out&#58; complete blood count&#44; biochemistry profile&#44; skeletal survey&#44; chest radiograph&#44; abdominal ultrasound&#44; and full physical examination&#46; All results were normal&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">What Is Your Diagnosis&#63;</span></p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Unifocal Langerhans cell histiocytosis &#40;LCH&#41; of the skin &#40;cutaneous eosinophilic granuloma&#41;&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">The lesion was surgically excised and the patient has been followed up for 18 months with no local or systemic recurrence&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0040" class="elsevierStylePara elsevierViewall">LCH is characterized by an infiltrate of cells with Langerhans cell phenotype and can affect various organs&#44; including the skin&#44; mucosas&#44; lymph nodes&#44; liver&#44; spleen&#44; bone&#44; digestive tract&#44; thymus&#44; and pituitary gland&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> According to some studies&#44; skin lesions are present in up to 50&#37; of patients in the initial stages of LCH and can therefore be decisive in establishing a diagnosis&#59; however&#44; because clinical presentation varies so widely&#44; LCH is often not suspected&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">LCH has traditionally been classified as follows&#58; eosinophilic granuloma &#40;single-site disease&#41;&#44; Hand-Sch&#252;ller-Christian disease &#40;chronic&#44; progressive&#44; multifocal disease&#41;&#44; and Letterer-Siwe disease &#40;acute&#44; diffuse disease with a poor prognosis&#41;&#46; In recent years&#44; however&#44; the Histiocyte Society has recommended classifying LCH by site and extent&#44; as follows&#58; type 1&#44; unifocal&#59; type 2&#44; multifocal without involvement of the liver&#44; spleen&#44; lungs&#44; or bone marrow&#59; type 3&#44; multifocal with involvement of the liver&#44; spleen&#44; lungs&#44; and&#47;or bone marrow&#44; or age less than 2 years&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">The typical&#44; classical LCH skin lesions are described as small&#44; purpuric&#44; infiltrated papules with a crusted or scaly surface on the scalp&#44; face&#44; flexures&#44; genital region&#44; and trunk&#46; However&#44; it should be noted that a wide variety of skin lesions of various morphologies can be found in LCH&#44;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> including the following&#58; blisters&#59; vesicles&#59; pustules&#59; single or multiple papules that can be skin-colored&#44; pink&#44; xanthomatous&#44; or purpuric&#59; tumors&#59; nail dystrophy&#59;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> and mucosal ulcerations&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> As a result&#44; the differential diagnosis is very extensive and includes Darier disease&#44; cutaneous candidiasis&#44; non-Langerhans cell histiocytosis&#44; intertrigo&#44; vasculitis&#44; urticaria pigmentosa&#44; sarcoidosis&#44; mycosis fungoides&#44; impetigo&#44; folliculitis&#44; herpes simplex&#44; and chickenpox&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">LCH is diagnosed histologically&#46; Skin lesions&#44; because of their accessibility&#44; are often the key to diagnosis&#46; Histologic examination reveals an infiltrate of histiocytoid cells with reniform nuclei that express CD1a and S-100&#46; Intracytoplasmic Birbeck granules are visible with electron microscopy&#46; In the case of our patient&#44; the late presentation of a supposed epidermal nevus at age 8 years prompted us to order the histologic study&#44; which led us to the correct diagnosis&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">Unifocal forms of LCH are usually found on bone&#44; most frequently the cranium and long bones&#46; Unifocal manifestation on the skin&#8212;as in the reported case&#8212;is rare&#46; These localized cases can be treated with nitrogen mustard&#44; psoralen&#8211;UV-A therapy&#44; surgery&#44; intralesional corticosteroids&#44; radiation therapy&#44; or thalidomide&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Prognosis is determined by the number of organs involved and will be worse if the liver&#44; lungs&#44; or bone marrow are affected&#46; Localized cutaneous forms of LCH have a good prognosis and rarely progress to multisystem disease&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a></p></span></span>"
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es en

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?