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The series published in North America and Europe have been limited, perhaps because this syndrome is rare in Caucasian population.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">2,3</span></a> We report the clinical characteristics and evolution of a series of 4 patients with VKHD diagnosed and monitored in internal medicine unit of a tertiary hospital over a period of 33 months.</p><p id="par0010" class="elsevierStylePara elsevierViewall">All patients were women and the median age at diagnosis was 33 years. Following the diagnostic guidelines of the American Society of Uveitis (2001),<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> one patient met the criteria for complete disease, 2 for incomplete disease, and one for probable disease.</p><p id="par0015" class="elsevierStylePara elsevierViewall">The evolution time of symptoms before the first consultation ranged from 2 to 21 days. The first symptom reported was headache in 3 patients. The remaining case began with impaired visual acuity (VA). Subsequently, all the patients developed progressive blurred vision; photophobia in 2 of them. As for extraocular symptoms, 2 patients had tinnitus, and one patient, sensorineural hearing loss.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Eye examination showed choroiditis with bilateral exudative retinal detachment in all patients. The VA to disease onset was 0.9 in one case, 0.2–0.5 in another, and “counting fingers” to 50<span class="elsevierStyleHsp" style=""></span>cm in the remaining 2.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Other symptoms during follow-up included hair graying plus hair loss in an individual, and skin hyperesthesia in another. None developed vitiligo.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Blood analysis was unremarkable in all patients. Lumbar puncture (LP) was performed in 3 patients, showing lymphomononuclear pleocytosis and negative microbiological test in CSF compatible with aseptic meningitis. On the other hand, magnetic resonance imaging (MRI) and computed tomography were normal.</p><p id="par0035" class="elsevierStylePara elsevierViewall">During admission all received systemic glucocorticoids, prednisone or equivalent, at doses of 1<span class="elsevierStyleHsp" style=""></span>mg/kg/day. Two patients who developed more significant impaired VA required boluses of methylprednisolone (250<span class="elsevierStyleHsp" style=""></span>mg/day) for 3 and 6 days respectively. No further immunosuppressants were required in any case.</p><p id="par0040" class="elsevierStylePara elsevierViewall">At discharge, all patients continued with prednisone 1<span class="elsevierStyleHsp" style=""></span>mg/kg/day on a decreasing regimen, which was held for four months in 2 patients, and 8 and 9 months in the remaining. At the end of treatment 3 subjects had a VA of 1 in the right eye and 0.9 in the left eye; the remaining patient had a VA of 1 in both eyes. By optical coherence tomography, ophthalmoscopy and control fluorescein angiography (FA) it was verified in all cases, complete healing of exudative retinal detachment, without occurrence of sequelae or relapses in the follow-up.</p><p id="par0045" class="elsevierStylePara elsevierViewall">The VKHD usually begins with neurological and eye symptoms common to other diseases, which, along with its low incidence, may cause a delayed diagnosis and the occurrence of complications or chronic and limiting visual sequelae.</p><p id="par0050" class="elsevierStylePara elsevierViewall">The median age at onset of the disease and the predilection for females is consistent with the data from other medical literature.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">4–6</span></a> Headache was the most common symptom, being a typical manifestation of the prodromal phase, characterized by meningeal symptoms.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> The exudative retinal detachment was the main ophthalmological finding, also reflected in other series with an incidence over 90%.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">5,6</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The 3 patients undergoing LP were found aseptic meningitis. This procedure might be avoided if all the signs and symptoms of the disease are shown, since the diagnosis might be established in a patient with indicative symptomatology and characteristic FA.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">7</span></a> Blood tests and imaging tests are often anodyne in this entity, only providing better diagnostic certainty. MRI is recommended, especially to differentiate VKHD from posterior scleritis.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">Glucocorticoids are the cornerstone of treatment, sometimes requiring methylprednisolone pulses to control the choroidal inflammation, as it occurred in 2 subjects in this series. Subsequently, all patients must follow a decreasing prednisone regimen, starting by 1<span class="elsevierStyleHsp" style=""></span>mg/kg/day for a minimum period of 6 months in order to obtain the best possible VA at the end of treatment.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">7</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">No subject in this series required second-line immunosuppressants, or reported complications or sequelae. In other studies, up to 43.75% of the patients required immunosuppressive agents (cyclosporine and azathioprine) to control inflammation, and 36.4% developed complications (cataracts, synechiae, etc.).<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">5</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">The good performance of the patients in this series might be due to the limited presence of factors associated with the development of complications.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">8</span></a> All subjects were female and developed the disease at an early age. In addition, only 2 had a serious illness onset (with “counting fingers” vision), and the time of symptomatology development was below one month before diagnosis.</p><p id="par0075" class="elsevierStylePara elsevierViewall">Comparing this series with other Spanish reports, we find similarity in the epidemiological spectrum, the initial symptomatology and the high frequency of exudative retinal detachment.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">5,6,9</span></a> As for the differences, healing occurs in 100% of patients, with no incidence of recurrence or chronic complications and no need for second-line immunosuppressants; all of this probably due to the small number of poor prognostic factors or high initial clinical suspicion.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Ortiz Balbuena J, Tutor de Ureta P, Rivera Ruiz E, Mellor Pita S. 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