Group of islands with snow
A 28-year-old male patient presented with complaints of repeated episodes of redness, watering, and pain for the past 4 and 10 years in the right eye (OD) and left eye (OS), respectively. He had no h/o trauma, joint pain, mouth ulcer, cough, or weight loss. He had been on multiple courses of oral steroids. On examination his best corrected vision was 6/24,N8 and 6/6,N6 in OD and OS respectively. Slit-lamp examination of both eyes (OU) revealed multiple macular grade opacities distributed centripetally in a radial manner with clear spaces in between, like an archipelago, from the inferior limbus to the center [Fig. 1a and b].
A week later, patient presented with OD scleral thinning temporally with a pus point. There were multiple areas of corneal subepithelial infiltration with clear spaces in between and decreased corneal sensation.
What is Your Next Step?
- a) Immune status
- b) Collagen workup
- c) Quantiferon TB gold
- d) Corneal and scleral scraping including PCR for HSV 1 and 2
- e) All of the above
A month later, he presented with 0.5 × 0.5 mm perforation with microleak with iris plugging, 2 mm from the limbus, with surrounding minimal cellular infiltration. There were no keratic precipitates, hypopyon, or active scleritis. Anterior chamber was shallow temporally and quiet [Fig. 1c]. A tenon patch graft [Fig. 1d], Fig. 1a long with the application of cyanoacrylate glue and a bandage contact lens, was performed for the perforation in the right eye, simulating a group of islands, some of which were snow covered [Fig. 1d, 1e]. The patient was started on oral acyclovir 500 mg five times per day along with topical steroids in tapering doses for 3 months.
Diagnosis- Archipelago keratitis
Correct answer- e) All of the above
Archipelago keratitis is an atypical form of herpes simplex viral epithelial keratitis arising from its centripetal progression. The migration of limbal epithelial cells to the center of the cornea facilitates the viral spread, resulting in this unique pattern. Alternatively, a centripetal propagation during viral reactivation could follow corneal nerves that are known to be oriented radially. The differential diagnosis of archipelago keratitis includes catarrhal staphylococcal marginal keratitis, Thygeson keratitis, and ocular rosacea.
In our case, features suggestive of viral etiology are multiple recurrences, response to oral antiviral therapy, centripetal pattern of arrangement, and resolution with steroid. Previous usage of oral antivirals, immune phase of the disease during the time when polymerase chain reaction (PCR) was performed, or inadequate samples could be accounted for the negative PCR. Archipelago keratitis can be recurrent and seen with induced immunosuppression with systemic or topical agents or due to acquired immune deficiency. Prompt initiation of topical antiviral therapy will aid in complete resolution and prevent scarring. At 3 years follow-up, tenon patch graft was well integrated with quiet anterior chamber, with no recurrences [Fig. 1f].
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The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
1. Gabison EE, Alfonsi N, Doan S, Racine L, Sultan G, Baudouin C, et al Archipelago keratitis:A clinical variant of recurrent herpetic keratitis?. Ophthalmology 2007;114:2000–5.
2. Matalia HP, Nandini C, Saishree M, Matalia J Archipelago keratitis. Indian J Ophthalmol 2019;67:555.