Vogt–Koyanagi–Harada Syndrome – Case report

We report a 28-year-old HIV-negative Ugandan woman with no known chronic illness. She presented to the eye clinic at Mulago National Referral Hospital (Kampala, Uganda) with a 2-week history of sudden onset blurring of vision in both eyes, especially in the mornings. This was preceded by a 1-week history of low-grade intermittent fevers, sore throat, running nose, and a nonproductive cough. These symptoms were attributed to a viral upper respiratory tract infection that was managed conservatively. She denied any history of headache, neck stiffness, floaters, excessive tearing, or reddening of the eyes but reported itching of both eyes. There was no history of chronic eye disease, trauma, or previous surgery on the eyes. There was a family history of blindness, which affected one of her four paternal uncles and his son who died under unclear circumstances. Notably, both relatives developed sudden loss of vision and had vitiligo-like skin patches.

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Multiple, symmetrical, “chalk-white”, sharply demarcated, nonscaly patches on both sides of the forehead and upper back, discrete scattered macules on upper and lower limbs, and the torso with intact sensation. Blanching was observed in these patches. White patches of hair involving the scalp hair, eyebrows, and eyelashes. No evidence of hair loss.

During this visit, eye examination revealed a best-corrected visual acuity (BCVA) of 6/36 in both eyes and intraocular pressures (IOP) of 10 mmHg and 11 mmHg in the left and right eye, respectively. On slit lamp examination, pigmented keratic precipitates (KPs), posterior synechiae, and vitreous inflammatory cells were noted. She was managed for an acute uveitis for which she was prescribed Maxitrol® eye drops (active ingredients being dexamethasone, neomycin, and polymyxin B) with minimal improvement after 2 weeks of treatment.

Two weeks later, she developed a sudden onset frontal headache which was throbbing in character and associated with reddening, tearing, and pain involving both eyes. She also reported transient episodes of visual loss but denied any history of neck pain, photophobia, phonophobia, loss of consciousness, or convulsions. During this time, she also noticed impaired hearing in the right ear with bilateral tinnitus, especially in quiet places. There was no associated discharge, dizziness, vertigo, nausea, or vomiting. She was initiated on oral prednisolone 10 mg daily for one week with reported improvement in the symptoms. Her vision improved bilaterally though it remained blurred with floaters. Hearing also improved bilaterally, but the tinnitus persisted.

Five weeks after the onset of the symptoms, she developed an intensely itchy and painful papular rash, which was generalised. This healed after a week but left some hypopigmentation around the face and trunk. There was also associated whitening of her scalp hair, eyebrows, and lashes. However, there was no hair loss/balding.

On clinical examination, she had multiple, “chalk-white”, sharply demarcated, nonscaly patches on both sides of the forehead and upper back, discrete scattered macules on the upper and lower limbs, and the torso with intact sensation. Blanching was observed in these patches. Patches of white hair involving the scalp hair, eyebrows, and eyelashes were also noted. She had no evidence of hair loss, and her fingernails and toenails were normal.

The thyroid gland was normal, and she was clinically euthyroid. Other systemic examination was unremarkable. Her vitals were within normal ranges: temperature = 36.7°C, BP = 120/80 mmHg, PR = 79 bpm, RR = 16 bpm, and SPO2 97% on ambient air.

Eight weeks after the onset of her disease, ocular examination revealed a BCVA of 6/36 in both eyes (same as previous), with IOP of 11 mmHg bilaterally. She had normal eyelids, with patches of white eyelashes. Her conjunctivae were normal. Keratic precipitates were noted on the endothelium of the cornea in both eyes. She had posterior synechiae, and the lenses were clear in both eyes. The inflammatory cells and flares were observed in the vitreous. Fundoscopy revealed a “sunset glow fundus”, serous retinal detachment, blurring of the optic disc margin, papillitis, and normal retinal vessels bilaterally. However, inflammatory cells (“snow balls”) were observed in the vitreous of the left eye.

Fundus photograph showing papillitis (P) with blurred optic disc, vitreous cells (VC), and retinal serous detachment (RSD) in the right eye and subretinal fibrosis (SF) in both eyes.

A diagnosis of VKH syndrome was considered, as possibly a complete VKH syndrome in the chronic stage. Full blood count, erythrocyte sedimentation rate, antinuclear antibody tests, and chest radiograph were normal. The Mantoux test and a venereal disease research laboratory test were all negative. Oral prednisolone was restarted at a dose of 15 mg daily for two weeks in addition to oral azathioprine at a dose of 50 mg daily for one month and possible refills for 6 months. She received counselling regarding her disease and on the irreversibility of her vitiligo and poliosis, which were of concern to her. She continues to attend follow-up clinic visits.

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