Case Report

Nummular keratopathy in a patient with Hyper-IgD Syndrome

Courtney L Kraus^1* and Susan M Culican^1,2

• * Corresponding author: Courtney L Kraus courtneykraus@me.com

Author Affiliations

¹ Washington University School of Medicine, 660 South Euclid Ave, Saint Louis, Missouri 63110, USA

² Department of Ophthalmology and Visual Sciences, Washington University School of Medicine, Saint Louis, Missouri 63110, USA

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Pediatric Rheumatology 2009, 7:14 doi:10.1186/1546-0096-7-14

Published: 5 August 2009

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 (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.