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Published on: December 15, 2011
Nummular keratopathy in a patient with Hyper-IgD Syndrome
Courtney L Kraus1, Susan M Culican
1Washington University School of Medicine, 660 South Euclid Ave, Saint Louis, Missouri 63110, USA. courtneykraus@me.com.
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.
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