Nummular keratopathy in a patient with Hyper-IgD Syndrome.

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 (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 to 20/20.

CONCLUSION

One possible form of end-organ damage associated with HIDS is vision threatening nummular keratopathy.