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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 (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.

SUBMITTER: Kraus CL

PROVIDER: S-EPMC2731058 | biostudies-literature | 2009 Aug

REPOSITORIES: biostudies-literature

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Nummular keratopathy in a patient with Hyper-IgD Syndrome.

Kraus Courtney L CL Culican Susan M SM

Pediatric rheumatology online journal 20090805

<h4>Purpose</h4>To report a case of recurrent nummular keratitis in a pediatric patient with Hyperimmunoglobulinemia D syndrome.<h4>Methods</h4>A retrospective chart review.<h4>Results</h4>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 immunosupp ...[more]

PMID: 19656398

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