Elsevier

Ophthalmology

Volume 107, Issue 4, April 2000, Pages 790-794
Ophthalmology

Original Articles
Progressive outer retinal necrosis caused by herpes simplex virus type 1 in a patient with acquired immunodeficiency syndrome

https://doi.org/10.1016/S0161-6420(99)00143-8Get rights and content

Abstract

Objective/background

To identify the etiologic agent of rapidly progressive outer retinal necrosis (PORN) in a 32-year-old man with acquired immunodeficiency syndrome (AIDS), who had retinitis developed from cytomegalovirus (CMV). Multiple yellowish spots appeared in the deep retina without evidence of intraocular inflammation or retinal vasculitis, diagnosed clinically as PORN. Death occurred after failure of multiple organs.

Design

Case report.

Methods

Both globes were taken at autopsy, fixed in formalin, and examined histopathologically and immunohistochemically to identify causative agents in the retinal lesions.

Main outcome measure

Immunohistochemistry.

Results

All layers of the retina were severely damaged and contained focal calcification. Cytomegalic inclusion bodies were found in cells in the damaged retina of the right eye. Immunohistochemical studies for herpesviruses revealed the presence of CMV antigens in the right retina at the posterior pole and herpes simplex virus type 1 (HSV-1)–specific antigen in the periphery of both retinas. No varicella-zoster virus (VZV) antigen was detected in either retina.

Conclusions

PORN has been described as a variant of necrotizing herpetic retinopathy, occurring particularly in patients with AIDS. Although the etiologic agent has been reported to be VZV, HSV-1 can be an etiologic agent.

Section snippets

Case presentation

A 32-year-old Japanese man, diagnosed with hemophilia B at the age of 3 years, had been treated with anticoagulant blood products. The patient was found to be infected with human immunodeficiency virus type 1 (HIV-1) at the age of 21. At the same time, he was diagnosed with AIDS at the C3 stage (CDC classification) because of the decreased CD4+ cell count, 98/μl, and the presence of esophagitis caused by Candida. After the diagnosis, he was treated with zidovudine and either didanosine or

Histologic and immunohistochemical methods

Both globes were obtained at autopsy and fixed in 10% formalin. The specimens first were cut horizontally to include the optic disc and then were cut perpendicular to the original plane. Tissue was processed routinely and embedded in paraffin. Some sections were stained with hematoxylin and eosin and others were stained by the periodic-acid–Schiff (PAS) method. Additionally, sections were examined by immunohistochemistry with a labeled streptavidin-biotin method (Dako, Kyoto, Japan) to detect

Microscopic findings including immunohistochemistry

Most retinal structures from the posterior pole to the periphery of both eyes were destroyed. In the periphery both eyes showed accompanying calcification. Retinal alterations were much more severe in the right eye than in the left. The outer retinal layer, however, remained in place at the posterior pole of the right eye, where retinal pigment epithelial cells (RPE) and Bruch’s membrane showed degeneration and lymphoid cells infiltrating into the choroid. Characteristic cytomegalic cells were

Discussion

PORN has been reported as a distinct form of ARN differing from acute necrotizing retinopathy caused by VZV in patients with AIDS.5 VZV antigen has been detected immunohistochemically in the outer retina in eyes showing PORN, and VZV is considered the usual etiologic agent.6 Engstrom et al11 have retrospectively studied 65 involved eyes from 38 patients and defined clinical criteria for PORN: multifocal lesions characterized by deep retinal opacification without granular borders, sometimes

Acknowledgements

The authors thank Ms. Yuko Sato and Ms. Noriko Sakurai for excellent technical assistance.

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  • Cited by (0)

    Supported by a grant-in-aid from Ministry of Health and Welfare, Japan (TS).

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