Vogt-Koyanagi-Harada disease in an 8-year-old boy

Abstract

Vogt-Koyanagi-Harada (VKH) disease is an autoimmune disease involving pigmented tissue in eyes, auditory system, skin, and central nervous system. The pathogenesis is a result of T cell lymphocyte reaction against melanocyte component, tyrosinase and tyrosinase-related protein. This disease uniquely affected pigmented race in Asia and native America, mostly women aged 20-50. We reported an 8-years-old boy complained for visual disturbance since 6 weeks prior admission. Two years earlier, the parents noted the patient eyes were looked red when photographed (suggesting a dilated pupil) preceded by whitened on the right forehead and nose and whitened hair, eyebrow and eyelashes. The examination showed a vitiligo and skin atrophy on right frontal and right nasal, poliosis on the eyebrows, eyelids and hair. The diameter of right pupil was 8 mm, with a paresis on 3rd, 4th, 6th, and 9th nerves. Fundus examination revealed sunset glow appearance. The visual acuity on the right eye: 2/60, left eye: 1-0. There's positive serology for antitoxoplasma IgG, anti Rubella IgG, anti CMV IgG. The patient was diagnosed with a VKH disease and then prescribed with methylprednisolone 1 mg/kg/day. The patient also had further follow up with dermatovenerologist and ophthalmologist. The eye examination revealed an improvement on right eye panuveitis, with a remaining keratic precipitate in the endothelium, and minimal flare and cell on the anterior chamber. ENT consultation revealed no ear inflammation or hearing disturbance.

Management of Bilateral Complicated Cataract in Convalescent Stage of Vogt- Koyanagi-Harada Syndrome

“…Kasus yang sama kemudian dilaporkan oleh Koyanagi pada tahun 1929, dan Harada pada tahun 1926 melaporkan pasien dengan uveitis, ablasio retina eksudatif dengan pleositosis pada cairan serebrospinal. 2,4 Di Indonesia, laporan kasus yang telah dipublikasikan antara lain pasien berusia 15 tahun pada tahun 1998 di Yogyakarta 5 dan pasien laki-laki berusia 8 tahun pada tahun 2011 di Rumah Sakit Hasan Sadikin Bandung. 4 Moorthy et al telah melaporkan katarak pada 40% pasien mereka di Amerika Serikat.…”

“…2,4 Di Indonesia, laporan kasus yang telah dipublikasikan antara lain pasien berusia 15 tahun pada tahun 1998 di Yogyakarta 5 dan pasien laki-laki berusia 8 tahun pada tahun 2011 di Rumah Sakit Hasan Sadikin Bandung. 4 Moorthy et al telah melaporkan katarak pada 40% pasien mereka di Amerika Serikat. 6 Terjadinya katarak pada uveitis dapat disebabkan oleh peradangan kronis dan efek dari pengobatan kortikosteroid jangka panjang.…”