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Woman presents with white retinal lesions

The patient reported several symptoms, including constant blurred vision, headache, vertigo, sore throat and mouth ulcers.

Issue: April 10, 2015

ByKendra A. Klein, MD

ByJay S. Duker, MD

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A 41-year-old Japanese-Caucasian woman was referred to the New England Eye Center for evaluation of retinal lesions in both eyes. Three weeks prior, she developed blurred vision, vertigo, headache, swollen neck glands, sore throat and malaise. She presented to an outside emergency department where she had a normal MRI of the brain and orbits and a positive throat culture for Streptococcus. She was seen by a consulting ophthalmologist who noted 20/200 vision in each eye with “white round patches surrounding the macula with areas of dot hemorrhages” in both eyes. She was treated with intravenous hydration and oral amoxicillin for presumed streptococcal pharyngitis and discharged with ophthalmology follow-up. She was an otherwise healthy woman without a significant medical, ocular or family history of disease.

On presentation to an outside retina specialist, the woman reported constant blurred vision at near and far, fatigue, subjective fevers and night sweats, sore throat, mouth ulcers, swollen cervical lymph nodes, vertigo, diarrhea and bifrontal headache. Best corrected visual acuity was 20/160 in the right eye and 20/80 in the left eye, without improvement on pinhole. IOP, extraocular motility and confrontation visual fields were normal, and her anterior segment examination was unremarkable. Posterior segment examination was notable for perifoveal white retinal infiltrates resembling cotton wool spots with small overlying dot hemorrhages in the macula of both eyes (Figures 1a to 1d). Fluorescein angiography revealed irregular-appearing foveal avascular zones with small vessel occlusions and discontinuity in the periphery of both eyes (Figures 1e to 1j). She was treated with 50 mg of oral prednisone daily for presumed bilateral, multifocal, obliterative small vessel disease with possible macular ischemia bilaterally. One week later, her exam and visual acuities were unchanged, and she was referred to the New England Eye Center for further evaluation.

On presentation to the New England Eye Center, many of her symptoms were beginning to subside, but her blurred vision, headache and vertigo were unchanged. BCVA was 20/200 and 20/80 in the right and left eyes, respectively, and anterior segment examination was unchanged. Posterior segment examination revealed improvement in the perifoveal retinal whitening and flame hemorrhages in both eyes and improved retinal whitening along the superior arcade in the left eye (Figures 2a to 2d). Fluorescein angiography revealed capillary leakage along the superotemporal arcade and macula, and capillary non-perfusion in the periphery in the left eye (Figures 2e and 2f). OCT of the macula revealed parafoveal hyperreflectivity of the inner retinal layers in both eyes (Figures 2g and 2h).

Figure 1. Fundus photography showing white retinal infiltrates around the fovea in the right eye (a) and along the fovea and superior arcade in the left eye (b) upon presentation to an outside retina specialist. Red-free photography demonstrating dot hemorrhages in the right eye (c) and dot hemorrhages with a flame hemorrhage temporally in the left eye (d). Fluorescein angiography revealed irregular-appearing foveal avascular zones with small vessel occlusions and discontinuity in the periphery of both eyes (e to j).

Images: Klein K, Duker JS

Figure 2. Fundus photography showing improved white retinal infiltrates around the fovea in the right eye (a) and along the fovea and superior arcade in the left eye (b). Red-free photography showed improved dot hemorrhages in the right eye (c) and dot and flame hemorrhages in the left eye (d). Fluorescein angiography showing capillary leakage along the superotemporal arcade and macula and capillary non-perfusion peripherally in the left eye (e to f). Spectral domain OCT of the macula revealed parafoveal hyperreflectivity of the inner retinal layers in the right eye (g) and left eye (h).

What is your diagnosis?

Bilateral white retinal lesions

The differential diagnosis of bilateral white retinal lesions with vasculitis without vitritis includes infectious etiologies including tuberculosis, syphilis, Lyme, toxoplasmosis, HIV retinopathy and rickettsiosis, and inflammatory etiologies including Behçet’s disease, systemic lupus erythematosus, polyarteritis nodosa and granulomatosis with polyangiitis. A full work-up was completed. HLA-B51 was positive. Negative testing included blood and urine cultures, basic metabolic profile, complete blood count, ANA, anti-dsDNA, SS-A/SS-B, RF, ANCA, anticardiolipin, protein C serologies, RPR, FTA-ABS, QuantiFERON Gold, serologic testing for HIV, West Nile virus, Babesia, Ehrlichia, Anaplasma, Lyme and Epstein-Barr virus. Based on these findings and the patient’s report of oral ulcers in the weeks before presentation, a diagnosis of Behçet’s disease was made. She was referred to rheumatology for a systemic work-up of Behçet’s disease and possible initiation of immunomodulatory agents. She was lost to follow-up.

Discussion

Behçet’s disease is a multisystem inflammatory disorder of unknown etiology, characterized by recurrent inflammatory attacks in multiple organ systems. Prevalence is greatest in the Mediterranean, Middle East and Far East, corresponding to the highest distribution of HLA-B51 antigen. Ocular disease occurs in about 35% to 50% of patients and typically occurs within the first 2 to 4 years of onset. It is the initial manifestation in 20% of cases and is bilateral in 60% to 100% of cases.

Uveitis, classically defined as chronic relapsing bilateral non-granulomatous panuveitis and retinal vasculitis (typically periphlebitis), is the most common ocular manifestation. Necrotizing retinitis, characterized by solitary or multifocal yellow-white retinal lesions that recur in a migratory pattern, is seen in 30% to 50% of patients. New lesions, even without signs of acute inflammation, should be considered signs of active disease. These often undergo spontaneous resolution with no residual chorioretinal scarring. The disease course is marked by recurrent inflammatory attacks and spontaneous resolution within days to weeks.

The literature on OCT in Behçet’s disease describes mostly chronic disease, as active vitritis precludes proper imaging in most of these patients. These studies have shown that chronically there is loss of the nerve fiber layer causing indentation of the inner retinal surface, disruption of the IS/OS junction and thinning of the choroid (presumably secondary to choroidal fibrosis). Our patient did not have vitritis, allowing imaging during acute disease. Interestingly, we found hyperreflectivity in the inner retinal layers, resembling images published by Sarraf and colleagues who described a new variant of acute macular neuroretinopathy called paracentral acute middle maculopathy. Using spectral domain OCT, the authors described a new variant, type 1 lesions, located at the outer plexiform/inner nuclear junction, implicating occlusion of the superficial capillary plexus located at the inner nuclear layer. The OCT findings in our patient suggested that the acute phase of Behçet’s disease may entail vasculitic occlusion of the superficial capillary plexus of the retina.