Asia Pac Allergy.  2011 Jul;1(2):98-103. 10.5415/apallergy.2011.1.2.98.

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

• Setiabudiawan B
• Karfiati F
• Ghrahani R
• Sapartini
• Sahril I

• KMID: 2397169
• DOI: http://doi.org/10.5415/apallergy.2011.1.2.98

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.

Keyword

Vogt-Koyanagi-Harada; Melanocyte; Visual disturbance; Methylprednisolone

MeSH Terms

Americas
Anterior Chamber
Asia
Atrophy
Autoimmune Diseases
Central Nervous System
Child*
Continental Population Groups
Endothelium
Eyebrows
Eyelashes
Eyelids
Female
Follow-Up Studies
Forehead
Hair
Hearing
Humans
Immunoglobulin G
Lymphocytes
Male*
Melanocytes
Methylprednisolone
Monophenol Monooxygenase
Nose
Otitis
Panuveitis
Parents
Paresis
Pupil
Rubella
Skin
Uveomeningoencephalitic Syndrome*
Visual Acuity
Vitiligo
Immunoglobulin G
Methylprednisolone
Monophenol Monooxygenase

Figure

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  Fig. 1 Poliosis on (A) right temporal, (B) the eyebrow, and (C) the eyelashes.

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  Fig. 2 (A) Showed vitiligo on right forehead and right nasal (black arrows); (B) assymetrical uvula indicated paresis on nervus IX.

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  Fig. 3 Paresis of 3rd, 4th, 6th cranial nerves.

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  Fig. 4 Photograph of the funduscopy showed retinal depigmentation (sunset glow appearance).

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  Fig. 5 Asymmetrical body.

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