Nummular keratopathy in a patient with Hyper-IgD Syndrome

Abstract Purpose To report a case of recurrent nummular keratitis in a pediatric patient with Hyperimmunoglobulinemia D syndrome.Methods A retrospective chart review.Results A 14-year-old boy with Hyperimmunoglobulinemia D syndrome redken shades eq 07m driftwood (HIDS) presented with photophobia and ocular irritation concomitant with disease exacerbation.He was found on exam to have significant nummular keratitis, which responded to a short course of topical steroids.Despite acute response to local immunosuppression, the patient had several recurrent attacks and eventually developed a large corneal scar and decreased vision.

After initiation of infliximab therapy his ocular sequelae improved dramatically and his vision returned grandpas best to 20/20.Conclusion One possible form of end-organ damage associated with HIDS is vision threatening nummular keratopathy.

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