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Pediatr Rheumatol Online J. 2009 Aug 5;7:14. doi: 10.1186/1546-0096-7-14.

Nummular keratopathy in a patient with Hyper-IgD Syndrome.

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Washington University School of Medicine, 660 South Euclid Ave, Saint Louis, Missouri 63110, USA.



To report a case of recurrent nummular keratitis in a pediatric patient with Hyperimmunoglobulinemia D syndrome.


A retrospective chart review.


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.


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

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