Sir,

Kyrieleis plaques were described in 1933 in ocular tuberculosis.1 They have been primarily described in association with infections of the retina, Toxoplasma gondii chorioretinitis being the most common.2 Other described associated causes include cytomegalovirus (CMV) retinitis, syphilitic retinitis, acute retinal necrosis (ARN) due to Herpes Simplex Virus-2, Varicella-Zoster Virus, and Rickettsia conorii infections.3, 4, 5 Orzalesi and Ricciardi6 suggested they are an immune response, resulting from deposition of immune cells and inflammatory debris in arterial walls. Others have debated this hypothesis as these plaques can persist despite resolution of the infection and treatment with steroids.7

Case report

A 56-year-old immunocompetent male presented with a 2-day history of decreased vision and floaters in the left eye. His past ophthalmic history included a single episode of Herpes Zoster Ophthalmicus in his right eye 3 months previously, which was successfully treated with 1 week of oral acyclovir.

Examination of the affected left eye revealed 3+ of anterior chamber cellular activity, with associated mutton fat keratic precipitates (KPs) on the inferior aspect of the corneal endothelium (Figure 1). There was no evidence of synechiae. Dilated examination of the posterior segment was hazy due to 2+ of vitritis and haze 1+ but despite this, 360 degrees of confluent retinal necrosis encircling the arcades was apparent. Kyrieleis plaques were present, without any evidence of sheathing of the retinal veins (Figure 2). Examination of the right eye was unremarkable, with no evidence of any inflammation. Best-corrected visual acuity was 0.1 Log Mar in the right and 0.5 in the left eye.

Figure 1
figure 1

Slit-lamp photo of the left anterior chamber on the day of presentation. It demonstrates 3+ of anterior chamber cellular activity and multiple mutton fat keratic precipitates on the inferior corneal endothelium.

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Figure 2
figure 2

Left eye fundus photograph demonstrating kyrieleis plaques within the arteries that do not extend outside the vessel. There is no significant sheathing or involvement of the retinal veins.

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Polymerase chain reaction of the aqueous tap was positive for HZV and negative for HSV, CMV, and Toxoplasma gondii DNA.

The patient was treated with 1 g intravenous Acyclovir three times/day for 5 days, followed by 2 g of oral Valacyclovir three times/day. After 7 days of antiviral treatment, concern over the safety of the optic nerve in the presence of an occlusive vasculitis meant that a course of 60 mg of oral Prednisone was introduced. This dose was maintained for 7 days.

Vitritis and haze significantly resolved after 10 days of treatment. Fluorescein angiography (FFA) demonstrated normal arterial filling and no evidence of active vasculitis. Therefore, prednisone was reduced to 40 mg/day. Kyrieleis plaques did not leak or have late staining on the FFA (Figure 3). The anterior uveitis and mutton fat KPs resolved over 2 weeks following initiation of treatment. Conversely, the Kyrieleis plaques increased in number and became confluent along the retinal arteries (Figure 4). Despite the visual acuity improved to Log Mar 0.2, the patient remained on a dose of 40 mg Prednisone daily. The plaques started to resolve over the following 4 weeks. Prednisone was tapered and discontinued gradually over a period of 8 weeks. At 3 months, Valaciclovir was reduced to 1 g three times/day and was eventually discontinued 14 weeks after initiation.

Figure 3
figure 3

Fundus fluorescein angiography (FFA) of the left eye, demonstrating normal retinal arterial filling. There is no fluorescein dye leakage in the macular area. The Kyrieleis plaques did not leak or have significant late staining of fluorescein dye.

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Figure 4
figure 4

Red-free fundus photograph (30°) of the left eye 2 weeks after initiation of treatment. Vitreous inflammation settled down, while the retinal lesion’s margins became more prominent, demarcated and some pigmentation started to appear in the margins.

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Written informed consent was obtained from the patient for publication of this case report and any accompanying images.

Comment

Contralateral ARN is well described is cases with Herpes Zoster Ophthalmicus. Second eye involvement occurs in approximately a third of patients, typically within 6 weeks,8 although fellow eye involvement decades following an initial infection has been described.9

Frances-Munoz et al3 report an association between Kyrieleis’ plaques and inflammation. In our case, the plaques increased as the vitritis and retinitis resolved. Interestingly, despite the high doses of steroids initiated in this case, the Kyrieleis’ plaques appeared to increase in number and spread around the optic disc while the overall inflammation was settling down. Eventually the plaques started to dissolve 4 weeks after presentation.

This case highlights the already known importance of examining the retina of the fellow eye when patients present with Herpes Zoster Ophthalmicus. In addition, the case provides further evidence that Kyrieleis plaques can potentially increase in size and number as inflammation settles down. The high-dose steroid treatment did not appear to have a role in limiting or reversing the development of these plaques.