VZV multifocal vasculopathy with ischemic optic neuropathy, acute retinal necrosis and temporal artery infection in the absence of zoster rash

Abstract

We describe a 54-year-old diabetic woman who developed ischemic optic neuropathy followed by acute retinal necrosis and multiple areas of focal venous beading. Vitreous fluid contained amplifiable VZV DNA but not HSV-1, CMV or toxoplasma DNA. The clinical presentation was remarkable for jaw claudication and intermittent scalp pain, prompting a temporal artery biopsy that was pathologically negative for giant cell arteritis, but notable for VZV antigen. The current case adds to the clinical spectrum of multifocal VZV vasculopathy. The development of acute VZV retinal necrosis after ischemic optic neuropathy supports the notion that vasculitis is an important additional mechanism in the development of VZV retinal injury.

Fig. 1

Fundus photo of the left eye demonstrates extensive retinal necrosis with intraretinal hemorrhage, overlying vitreitis (long arrows) and focal beading in multiple retinal veins (short arrows).

Fig. 2

Cross-sections of the temporal artery showed mild intimal thickening (short arrows) and an intact internal elastic lamina (long arrow) (A, H&E; B, Van Gieson stain, respectively; magnification 100X). Hyalinazation of vasa vasorum arterioles was noted (C, long arrows), consistent with diabetic vasculopathy. No evidence of inflammation was noted, particularly in the adventitial connective tissue or in association with vasa vasorum or vasa nervosum structures (H&E, magnification 400X. CD45 immunohistochemistry, as previously described [11], confirmed the absence of an inflammatory cell infiltrate (not shown).

Fig. 3

The left temporal artery in a patient with multifocal vasculopathy was analyzed for the presence varicella zoster virus (VZV) antigen as previously described [1]. A positive control cadaveric cerebral artery 14 days after VZV infection in vitro (A, pink color, arrows). VZV antigen was seen in the adventitia of the left temporal artery of the subject after staining with anti-VZV antibody (B, pink color, arrows), but not after staining adjacent sections with anti-HSV antibody (C) or normal rabbit serum (D). Magnification 200X.