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Ophthalmological examination revealed a marked loss of visual acuity in both eyes&#59; anterior pole biomicroscopy revealed Tyndall phenomenon with posterior synechiae &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Nonscarring diffuse alopecia was also observed on the scalp&#44; together with poliosis of the right eyebrow &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#44; A and B&#41;&#46; The results of the blood workup&#8212;complete blood count&#44; biochemistry&#44; clotting&#44; protein analysis&#44; antibodies to antinuclear antigen and extractable nuclear antigen&#44; serology for syphilis and viruses &#40;hepatitis A&#44; B&#44; and C viruses&#59; human immunodeficiency virus&#59; Epstein-Barr virus&#59; cytomegalovirus&#41;&#44; and thyroid hormones&#8212;were negative or normal&#46; Cerebral magnetic resonance imaging ruled out meningeal or encephalic disease&#46; Histocompatibility testing was positive for HLA-DR4 and HLA-DR53&#46; These findings enabled a diagnosis of Vogt-Koyanagi-Harada disease to be confirmed&#46; Treatment with intravenous methylprednisolone &#40;1<span class="elsevierStyleHsp" style=""></span>g&#41; was prescribed for 3 days&#44; with subsequent oral administration tapered over 12 weeks&#46; The patient&#39;s clinical condition improved considerably&#44; not only in terms of systemic manifestations&#44; but also with complete resolution of the alopecia&#46; However&#44; the new hair remained whitish-gray in color &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Vogt-Koyanagi-Harada disease&#44; which was first reported in 1951&#44; is a rare systemic inflammatory disorder mediated by T lymphocytes acting against pigmented cells in the uvea&#44; skin&#44; inner ear&#44; and leptomeninges&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">It has an incidence of 6&#46;5 cases per million and is more common in Hispanic and Native American individuals and Asian women aged between 20 and 50 years&#46; Although its etiology is unknown&#44; immunogenetic analysis suggests a strong association with some HLA antigens &#40;DR4&#44; DRB1&#44; and DR53&#41; and genes of the tyrosinase family that cause susceptibility to the disease&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> It is associated with various infectious diseases and other disorders whose pathogenic mechanisms are of immunological origin&#44; such as autoimmune thyroid disease&#44; psoriasis&#44; linear immunoglobulin A bullous dermatosis&#44; and inflammatory vitiligo as a consequence of the destruction of melanocytes by cytotoxic CD8<span class="elsevierStyleSup">&#43;</span> T lymphocytes&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#8211;4</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Diagnosis is based essentially on clinical manifestations&#44; as there are no specific confirmatory tests&#44; and the diagnostic criteria include dermatologic findings &#40;alopecia&#44; poliosis&#44; and vitiligo&#41; &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#41;&#46; Typical extracutaneous clinical manifestations include disorders of the eyes &#40;bilateral granulomatous uveitis&#44; retinal detachment&#44; and loss of vision&#41; and neurological and auditory conditions &#40;headache&#44; aseptic meningitis&#44; vertigo&#44; nystagmus&#44; and hypoacusis&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Alopecia can be seen in 70&#37; of cases within weeks or months after the onset of ocular symptoms&#46; It resolves completely after a variable period&#46; Alopecia and the poliosis traditionally reported by ophthalmologists were recently considered to be alopecia areata with subsequent growth of white hair&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> Vitiligo affects almost half of all cases&#59; it is often symmetrical and the pigmentary changes may be permanent&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Early high-dose systemic corticosteroids are the treatment of choice in patients with active Vogt-Koyanagi-Harada disease&#46; In cases of recurrent or corticosteroid-refractory disease&#44; other immunosuppressive agents such as ciclosporin&#44; cyclophosphamide&#44; or azathioprine can be added to the treatment&#46; A favorable response has been reported with adalimumab and rituximab in refractory cases&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> Prognosis is relatively benign&#44; and although the most common complications are visual&#44; total blindness is now rare&#46; Patients may also experience personality disorders&#44; psychosis&#44; and deafness&#44; as occurred in the Spanish painter Francisco de Goya when creating his famous black paintings&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a></p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Hern&#225;ndez-Bel P&#44; et al&#46; Enfermedad de Vogt-Koyanagi-Harada&#46; Una entidad poco conocida para el dermat&#243;logo&#46; Actas Dermosifiliogr&#46; 2013&#59;104&#58;529-31&#46;</p>"
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Tyndall phenomenon with the presence of posterior synechiae characteristic of anterior uveitis&#46;</p>"
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                  \t\t\t\t"><span class="elsevierStyleItalic">Major Criteria</span>&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>1&#46; No history of penetrating eye trauma or surgery before the episode of uveitis&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>2&#46; No clinical or analytical evidence suggestive of other eye diseases&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>3&#46; Bilateral ocular involvement &#40;choroiditis&#44; uveitis&#44; inflammatory vitreous reaction&#44; serous retinal detachment&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>4&#46; Neurological and auditory findings &#40;meningism&#44; tinnitus&#44; pleocytosis in cerebrospinal fluid&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>5&#46; Dermatologic findings &#40;alopecia&#44; poliosis&#44; vitiligo&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>6&#46; Hypoxia&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>7&#46; Central nervous system depression&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>9&#46; Tachycardia &#40;&#62;<span class="elsevierStyleHsp" style=""></span>120 beats per minute&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>10&#46; Fever &#40;temperature &#62;<span class="elsevierStyleHsp" style=""></span>39<span class="elsevierStyleHsp" style=""></span>&#176;C&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>11&#46; Unexplained anemia&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>12&#46; Thrombocytopenia &#40;platelet count &#60;<span class="elsevierStyleHsp" style=""></span>150<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>10<span class="elsevierStyleSup">9</span>&#47;L&#41;&nbsp;\t\t\t\t\t\t\n
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        "texto" => "<p id="par0040" class="elsevierStylePara elsevierViewall">We are grateful to our colleagues from the dermatology and ophthalmology departments who participated in the diagnosis and management of the patients&#46;</p>"
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Case and Research Letter
Vogt-Koyanagi-Harada Disease: A Disorder Unfamiliar to Dermatologists
Enfermedad de Vogt-Koyanagi-Harada. Una entidad poco conocida para el dermatólogo
P. Hernández-Bela,
Autor para correspondencia
pablohernandezbel@hotmail.com

Corresponding Author.
, J. Monterob, L. Hernández-Belb, A. Torrijos-Aguilarc
a Servicio de Dermatología, Consorcio Hospital General Universitario de Valencia, Valencia, Spain
b Servicio de Oftalmología, Consorcio Hospital General Universitario de Valencia, Valencia, Spain
c Servicio de Dermatología, Hospital Provincial de Castellón, Castellón, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Vogt-Koyanagi-Harada disease is a rare condition that is characterized by bilateral uveitis&#44; meningitis&#44; auditory symptoms&#44; and skin disorders such as vitiligo&#44; poliosis&#44; and alopecia&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> This report aims to draw attention to a condition that is widely discussed in the literature yet unfamiliar to dermatologists&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">We present the case of a 37-year-old man from Bolivia who was seen in the emergency department with vertigo&#44; headache&#44; eye pain&#44; and loss of vision in both eyes that had begun a week earlier&#46; Three months later he was referred to the dermatology department with rapid-onset alopecia of the scalp&#46; Physical examination revealed horizontal rotatory nystagmus at rest and on looking upward and to the right&#46; Ophthalmological examination revealed a marked loss of visual acuity in both eyes&#59; anterior pole biomicroscopy revealed Tyndall phenomenon with posterior synechiae &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Nonscarring diffuse alopecia was also observed on the scalp&#44; together with poliosis of the right eyebrow &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#44; A and B&#41;&#46; The results of the blood workup&#8212;complete blood count&#44; biochemistry&#44; clotting&#44; protein analysis&#44; antibodies to antinuclear antigen and extractable nuclear antigen&#44; serology for syphilis and viruses &#40;hepatitis A&#44; B&#44; and C viruses&#59; human immunodeficiency virus&#59; Epstein-Barr virus&#59; cytomegalovirus&#41;&#44; and thyroid hormones&#8212;were negative or normal&#46; Cerebral magnetic resonance imaging ruled out meningeal or encephalic disease&#46; Histocompatibility testing was positive for HLA-DR4 and HLA-DR53&#46; These findings enabled a diagnosis of Vogt-Koyanagi-Harada disease to be confirmed&#46; Treatment with intravenous methylprednisolone &#40;1<span class="elsevierStyleHsp" style=""></span>g&#41; was prescribed for 3 days&#44; with subsequent oral administration tapered over 12 weeks&#46; The patient&#39;s clinical condition improved considerably&#44; not only in terms of systemic manifestations&#44; but also with complete resolution of the alopecia&#46; However&#44; the new hair remained whitish-gray in color &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Vogt-Koyanagi-Harada disease&#44; which was first reported in 1951&#44; is a rare systemic inflammatory disorder mediated by T lymphocytes acting against pigmented cells in the uvea&#44; skin&#44; inner ear&#44; and leptomeninges&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">It has an incidence of 6&#46;5 cases per million and is more common in Hispanic and Native American individuals and Asian women aged between 20 and 50 years&#46; Although its etiology is unknown&#44; immunogenetic analysis suggests a strong association with some HLA antigens &#40;DR4&#44; DRB1&#44; and DR53&#41; and genes of the tyrosinase family that cause susceptibility to the disease&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> It is associated with various infectious diseases and other disorders whose pathogenic mechanisms are of immunological origin&#44; such as autoimmune thyroid disease&#44; psoriasis&#44; linear immunoglobulin A bullous dermatosis&#44; and inflammatory vitiligo as a consequence of the destruction of melanocytes by cytotoxic CD8<span class="elsevierStyleSup">&#43;</span> T lymphocytes&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#8211;4</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Diagnosis is based essentially on clinical manifestations&#44; as there are no specific confirmatory tests&#44; and the diagnostic criteria include dermatologic findings &#40;alopecia&#44; poliosis&#44; and vitiligo&#41; &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#41;&#46; Typical extracutaneous clinical manifestations include disorders of the eyes &#40;bilateral granulomatous uveitis&#44; retinal detachment&#44; and loss of vision&#41; and neurological and auditory conditions &#40;headache&#44; aseptic meningitis&#44; vertigo&#44; nystagmus&#44; and hypoacusis&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Alopecia can be seen in 70&#37; of cases within weeks or months after the onset of ocular symptoms&#46; It resolves completely after a variable period&#46; Alopecia and the poliosis traditionally reported by ophthalmologists were recently considered to be alopecia areata with subsequent growth of white hair&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> Vitiligo affects almost half of all cases&#59; it is often symmetrical and the pigmentary changes may be permanent&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Early high-dose systemic corticosteroids are the treatment of choice in patients with active Vogt-Koyanagi-Harada disease&#46; In cases of recurrent or corticosteroid-refractory disease&#44; other immunosuppressive agents such as ciclosporin&#44; cyclophosphamide&#44; or azathioprine can be added to the treatment&#46; A favorable response has been reported with adalimumab and rituximab in refractory cases&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> Prognosis is relatively benign&#44; and although the most common complications are visual&#44; total blindness is now rare&#46; Patients may also experience personality disorders&#44; psychosis&#44; and deafness&#44; as occurred in the Spanish painter Francisco de Goya when creating his famous black paintings&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a></p></span>"
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          "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A&#44; Diffuse rapid-onset alopecia on the scalp&#46; B&#44; Poliosis of the left eyebrow&#46;</p>"
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          "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Complete resolution of alopecia after treatment&#44; with growth of whitish-gray hair&#46;</p>"
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                  \t\t\t\t"><span class="elsevierStyleItalic">Major Criteria</span>&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>1&#46; No history of penetrating eye trauma or surgery before the episode of uveitis&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>2&#46; No clinical or analytical evidence suggestive of other eye diseases&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>3&#46; Bilateral ocular involvement &#40;choroiditis&#44; uveitis&#44; inflammatory vitreous reaction&#44; serous retinal detachment&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>4&#46; Neurological and auditory findings &#40;meningism&#44; tinnitus&#44; pleocytosis in cerebrospinal fluid&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>5&#46; Dermatologic findings &#40;alopecia&#44; poliosis&#44; vitiligo&#41;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>6&#46; Hypoxia&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>7&#46; Central nervous system depression&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>8&#46; Petechiae&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span>&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleItalic">Minor Criteria</span>&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>9&#46; Tachycardia &#40;&#62;<span class="elsevierStyleHsp" style=""></span>120 beats per minute&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>10&#46; Fever &#40;temperature &#62;<span class="elsevierStyleHsp" style=""></span>39<span class="elsevierStyleHsp" style=""></span>&#176;C&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>11&#46; Unexplained anemia&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span>12&#46; Thrombocytopenia &#40;platelet count &#60;<span class="elsevierStyleHsp" style=""></span>150<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>10<span class="elsevierStyleSup">9</span>&#47;L&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
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          "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Diagnostic Criteria of Vogt-Koyanagi-Harada Disease&#46;</p>"
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      ]
    ]
    "bibliografia" => array:2 [
      "titulo" => "References"
      "seccion" => array:1 [
        0 => array:2 [
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          "bibliografiaReferencia" => array:8 [
            0 => array:3 [
              "identificador" => "bib0005"
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                        ]
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                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
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                      "Revista" => array:6 [
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            1 => array:3 [
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                      "autores" => array:1 [
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            ]
            2 => array:3 [
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                      "autores" => array:1 [
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                            1 => "N&#46; Mizuno"
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                        ]
                      ]
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                  ]
                ]
              ]
            ]
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                      "autores" => array:1 [
                        0 => array:2 [
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                            0 => "D&#46; Tsuruta"
                            1 => "T&#46; Hamada"
                            2 => "H&#46; Teramae"
                            3 => "H&#46; Mito"
                            4 => "M&#46; Ishii"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1067/mjd.2001.110879"
                      "Revista" => array:6 [
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                      "autores" => array:1 [
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                            4 => "S&#46; Ohno"
                            5 => "L&#46; Arellanes-Garc&#237;a"
                          ]
                        ]
                      ]
                    ]
                  ]
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                    0 => array:2 [
                      "doi" => "10.1016/s0002-9394(01)00925-4"
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                      "titulo" => "Vogt-Koyanagi-Harada syndrome&#58; Association with alopecia areata"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
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                            0 => "W&#46;M&#46; Haque"
                            1 => "M&#46;R&#46; Mir"
                            2 => "S&#46; Hsu"
                          ]
                        ]
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                  ]
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                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:3 [
                            0 => "R&#46; Dolz-Marco"
                            1 => "R&#46; Gallego-Pinazo"
                            2 => "M&#46; D&#237;az-LLopis"
                          ]
                        ]
                      ]
                    ]
                  ]
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              ]
            ]
            7 => array:3 [
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              "etiqueta" => "8"
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                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "The black paintings and the Vogt-Koyanagi-Harada syndrome"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "L&#46;M&#46; Vargas"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
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    ]
    "agradecimientos" => array:1 [
      0 => array:4 [
        "identificador" => "xack628115"
        "titulo" => "Acknowledgments"
        "texto" => "<p id="par0040" class="elsevierStylePara elsevierViewall">We are grateful to our colleagues from the dermatology and ophthalmology departments who participated in the diagnosis and management of the patients&#46;</p>"
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Información del artículo
ISSN: 15782190
Idioma original: Inglés
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2013 Agosto 5 3 8
2013 Julio 2 4 6
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¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

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