[Vogt-Koyanagi-Harada Disease: clinical features, therapy and long-term visual outcome in a Caucasian and African population]. / Le syndrome de Vogt-Koyanagi-Harada: aspects cliniques, traitement et suivi à long terme dans une population caucasienne et africaine.

PURPOSE: Seventeen consecutive cases of Vogt-Koyanagi-Harada (VKH) disease were studied to determine their clinical profile. METHODS: This was a retrospective study of 17 cases, in a white and African population. RESULTS: The sex ratio (female/male) was 1.6. Mean age was 37.65 +/- 10.2 years. Eight patients were Caucasian (47%), and seven were from North Africa (41%), and two were black Africans (12%). Eleven patients were referred during the acute stage, and six patients secondarily. All patients had bilateral ocular involvement. Panuveitis with retinal serous detachment was the most frequent presentation (88%). Extraocular signs were found in 87% of the cases. Initial visual acuity was 0.29 +/- 0.36, and final visual acuity was 0.78 +/- 0.3. Patients seen during the acute stage were treated with general corticotherapy. Immunosuppressive agents were given in 56% of the cases. CONCLUSIONS: Vogt-Koyanagi-Harada disease, in a Caucasian and African population, has a presentation close to that of the Japanese population. However, cutaneous signs are much rarer. Visual prognosis was generally favorable.

[Loss of visual acuity due to choroidal ischemia in Fabry's disease]. / Baisse d'acuité visuelle par ischémie choroïdienne chez un patient atteint de la maladie de Fabry.

We report a case of a 21-year-old man with Fabry's disease who presented with a sudden decrease in visual acuity to 20/200 in the left eye. Pale areas with a lobular choroidal distribution were seen on fundus examination. No retinal vascular causes were found on further evaluation. With anticoagulation treatment, the patient's subsequent course was good, with visual recovery to 20/25 and normalization of the funduscopic appearance. Recovery of both visual acuity and the pale, lobular areas suggested a choroidal etiology, probably ischemic because of the sudden onset. Choroidian ischemia is therefore a cause of visual acuity loss in Fabry's disease, so far not described in the literature.