Stromal Keratitis in a Patient with Congenital Hypogammaglobulinemia

Abstract

Purpose: We report a case of stromal keratitis in a patient with congenital hypogammaglobulinemia. Case summary: A 15-year-old boy presented with decreased visual acuity in the right eye. He had been diagnosed with congenital hypogammaglobulinemia at about 16 months of age and had received regular doses of intravenous immunoglobulin. The best-corrected visual acuity of the right eye was 0.02 and the intraocular pressure 11 mmHg. On anterior segment examination, thinning combined with stromal infiltration of the paracentral cornea was evident, but no epithelial defect was apparent. We scheduled detailed systemic examinations and laboratory investigations to rule out infectious keratitis. His serum immunoglubulin G (IgG) level was 328.9 mg/dL, thus less than that 3 months priorly (434.8 mg/dL). The lesion did not improve after prescription of topical antibiotics and steroid. The serum IgG level gradually increased to 394.4 mg/dL after immunoglobulin administration, and the corneal infiltration gradually decreased. After 5 months of treatment, the serum IgG levels ranged between 480 and 530 mg/dL; we noted no recurrence or worsening of the corneal lesion. Conclusions: We report a case of stromal keratitis in a patient with congenital hypogammaglobulinemia; we prescribed intravenous immunoglobulin.