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VKH Syndrome

Introduction

VKH primarily affects the eye, skin, ears, and nervous system.
- Will explore the posterior eye aspect.
- Immune response to specific melanocytes, where T-cells begin to attack it
- Genetically susceptible individuals, such as impacted HLA-DR4, will suffer viral infections
- Viral infections are typically the trigger to VKH beginning
- Characterised by patchy depigmentation of the skin and alopecia (hair loss)
- Also associated with hearing loss and inner ear problems

Dead Giveaways

Acute Phase:

  • Characterised by choroidal thickening, disc hyperaemia and oedema, exudative retinal detachment, vitritis

    From the Eye Wiki. Shows the choroidal thickening, disc hyperaemia and oedema
    From the Eye Wiki. Shows the choroidal thickening, disc hyperaemia and oedema
    Shows the exudative retinal detachment, with the choroidal thickening and folding. From Urzua C.A. et al. 2022. Each folded line represents are potential area of detachment
    Shows the exudative retinal detachment, with the choroidal thickening and folding. From Urzua C.A. et al. 2022. Each folded line represents are potential area of detachment

Chronic Phase:

  • Characterised by the Sunset Fundus, occuring due to complete depigmentation of the choroid.

    From Gupta A et al. 2024. Shows the sunset glow appearance and hyperaemic disc of the chronic VKH phase
    From Gupta A et al. 2024. Shows the sunset glow appearance and hyperaemic disc of the chronic VKH phase

diagnostic features

Refer to Anterior Eye Diseases

Acute Phase:

  • Presents with bilateral acute granulomatous panuveitis

  • Poor control and treatment will lead to chronic VKH, which has a very poor visual outcome

2025, made by Eric Qin. UNSW. SOVS

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