Sir,We examined a 17-year-old female, who presented with complaints of a decrease in vision and metamorphopsia in both the eyes. On examination, her visual acuity was 6/18 in right eye and 6/24 in the left eye. Slit lamp examination showed the presence of keratic precipitates in both the eyes along with anterior chamber cells, flare, and cells in anterior vitreous. On fundus examination, there was presence of small, multiple, yellow choroidal lesions with presence of disc edema and exudative retinal detachment. B-scan confirmed retinal detachment and also showed choroidal thickening. A presumptive diagnosis of Vogt-Koyanagi-Harada syndrome was made. Patient was evaluated with blood investigations which showed raised erythrocytic sedimentation rate and negative venereal disease research laboratory test. Patient was treated with high-dose systemic corticosteroids (1.5 mg/kg) which resulted in complete remission of the disease and patient reached normal visual acuity of 6/6 in both eyes. Patient was on follow-up and was under oral azathioprine and corticosteroid for 18 months.After 27 years, the patient presented with complaints of pain in the left eye, which on examination showed the presence of necrotizing scleritis [Figure 1]. Collagen work up including a rheumatoid factor, ANA, C-ANCA, and P-ANCA were negative. Patient was started on oral azathioprine and prednisolone. The visual acuity in right eye is maintained at 6/6 while left eye had a vision of 3/60 due to the development of chronic macular edema following hypotony. Fundus evaluation revealed sunset glow appearance. The patient is coming for regular follow-up and is improving with treatment.
Figure 1
Slit lamp photograph of left eye showing necrotizing scleritis
Slit lamp photograph of left eye showing necrotizing scleritisNecrotizing scleritis can be associated with systemic connective tissue, including rheumatoid arthritis, Wegener granulomatosis, systemic lupus erythematosus, and sarcoidosis. Patients with scleritis may present either with a known underlying disorder like rheumatoid arthritis, or it can present de novo in the absence of any known systemic illness.[1]There had been reports of the development of Vogt-Koyanagi-Harada in patients of scleritis or concurrent development of bilateral posterior scleritis and Vogt-Koyanagi-Harada disease in a patient.[23] However, in our patient, scleral inflammation was observed following Vogt-Koyanagi-Harada disease and that too after an interval of 27 years. To our knowledge, this is the first report of the development of necrotizing scleritis in a case of Vogt-Koyanagi-Harada disease with a time gap of 27 years.
Figure 1