Recurrent keratoconjunctivitis in X-linked agammaglobulinemia: a microbiome analysis: a case report

Purpose: To describe recurrent keratoconjunctivitis in a patient with X-linked agammaglobulinemia (XLA) and report the findings of microbiome analysis.Case summary: A 15-year-old male with XLA presented with photophobia, epiphora, redness, and reduced vision in the right eye. Examination revealed corneal infiltration, neovascularization (NV), and thinning in the right eye, with multiple subepithelial opacities in the left. The corneal lesions improved transiently with topical corticosteroids and antibiotics but repeatedly worsened approximately 1 week before each scheduled monthly intravenous immunoglobulin (IVIG) infusion. Approximately 23 months later, corneal NV, central opacity, and thinning also developed in the left eye. Cultures of both conjunctivae yielded Haemophilus influenzae; a right eyelid culture also yielded Staphylococcus epidermidis. Conjunctival microbiome analysis confirmed H. influenzae and detected additional organisms—Mycoplasma amphoriforme, Streptococcus pneumoniae, and Staphylococcus aureus.Conclusion: In this patient with XLA, topical therapy provided only transient improvement in recurrent keratoconjunctivitis. Exacerbations were temporally associated with the presumed trough period before each scheduled IVIG infusion. Conjunctival microbiome analysis detected additional organisms not identified by bacterial culture.