Bilateral viral retinitis in a patient with immune deficiency because of purine nucleoside phosphorylase deficiency.

BACKGROUND

Progressive retinal outer necrosis is a devastating viral necrotizing retinitis, which affects predominantly immunocompromised patients. We report a case in which the ocular findings led to a search for an underlying immune deficiency, established as congenital purine nucleoside phosphorylase deficiency.

METHODS

Retrospective, interventional case report.

RESULTS

We describe the clinical features and management of the disease.

CONCLUSION

This is the first report of bilateral varicella zoster virus retinitis with underlying purine nucleoside phosphorylase deficiency. It highlights the importance of thorough investigation in apparent immunocompetent patients presenting with viral retinitis, as this may indicate an undiagnosed primary immunodeficiency.