Presumed autoimmune retinopathy following chickenpox; a case report

To report a case of presumed autoimmune retinopathy in a patient who had been diagnosed with chickenpox immediately prior to symptom onset.

This is a retrospective case report with fundus autofluorescence and spectral-domain optical coherence tomography.

A 29-year-old immunocompetent man presented with a two-month history of photopsia and a bluish light predominantly in the left eye with onset immediately following a diagnosis of chickenpox. He subsequently developed blurring of vision in his left eye with spectral-domain optical coherence tomography showing disruption and loss of ellipsoid layer in a perivascular pattern in both eyes with fundus autofluorescence demonstrating an abnormal increase in autofluorescence predominately around the vascular arcades. Autoimmune retinopathy was suspected on the basis of the clinical presentation, electrophysiology, absence of fundus lesions, retinal degenerations or dystrophies, or intraocular inflammation and temporal association with varicella infection. Antiretinal antibody testing with Western blot and immunohistochemistry were reported to be negative, however, given the consistent evidence from all other aspects of workup and investigation the patient was commenced on a trial of prednisone. This resulted in marked reduction in photopsia and expansion of visual fields.

The diagnosis of autoimmune retinopathy is ultimately clinical in nature. Despite the absence of positive retinal autoantibodies, a diagnosis of autoimmune retinopathy was made based upon consistent evidence from all other aspects of workup and investigation. This allowed prompt treatment with immunosuppressive agents with subsequent stabilization of this patient’s visual function.