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Sir,

The aetiology of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) remains a subject of debate. Our patient represents the second reported case of APMPPE associated with adenoviral infection.1

Case report

A 37-year-old Caucasian male presented with acute bilateral blurring of vision. He also reported a 3-week period of prodromal general malaise with symptoms of myalgia, arthralgia, headache and nonproductive cough. He had no significant past history and was not taking any regular medication. Family history was negative.

Visual acuities at presentation were right eye (RE) 6/24 and left eye (LE) 6/36. Anterior and vitreous chambers were quiet. Fundoscopy showed subtle perifoveal pallor. The next day visual acuities had fallen to 6/60 in both eyes. Ocular examination revealed slightly injected bulbar conjunctivae and <1+ anterior chamber cells without vitritis. Multiple, pale, greyish fundal lesions were now present in the postequatorial regions, densest at the posterior poles. These lesions were up to one disc diameter in size with mild overlying retinal oedema. A clinical diagnosis of APMPPE was made.

Plasma viscosity was 1.77 mPa s (normal range 1.5–1.72) and C-reactive protein, 18 mg/l (normal <5). Serum angiotensin converting enzyme, antinuclear antibody, antineutrophil cytoplasmic antibody and chest X-ray were normal or negative. No treatment was prescribed.

By day 4, visual acuities were RE CF and LE 6/60. Intravenous fundal fluorescein angiography demonstrated changes typical of APMPPE, namely early hypofluorescence followed by late staining of the fundal lesions (Figure 1). In view of the severity of visual loss, the patient was commenced on a 7-day course of oral prednisolone 40 mg daily. Visual acuities subsequently improved, RE 6/60 and LE 6/18 on day 7.

Figure 1
figure 1

Fluorescein angiography, day 4. Top: early arterio-venous phase, showing hypofluorescence of placoid lesions. Bottom: late staining of placoid lesions at 6 min.

Full size image

On day 12, he presented with a marked bilateral follicular conjunctivitis. Viral conjunctivitis was diagnosed and no topical treatment was given. Conjunctival swabs taken at this stage later identified adenovirus (direct immunofluorescence of cultured cell suspension). Bacterial and chlamydial swabs were negative. Later, the conjunctivitis worsened with the formation of conjunctival pseudomembrane, bilateral punctate corneal epitheliopathy and eyelid oedema. Subepithelial corneal opacities were noted on day 27.

On day 18, visual acuity in the LE had improved to 6/12 but in the RE, it remained 6/60. Fundal lesions were fading, with early pigmentary deposition. By day 39, retinal pigmented epithelium scars had formed including foveal involvement in the RE. Visual recovery continued in the LE with a final visual acuity of 6/5 on day 140, while the RE remained limited at 6/60.

Comment

This is the second reported case of proven adenovirus occurring simultaneously with the acute phase of APMPPE and lends support to a respiratory virus being implicated in the aetiology of this rare condition.1 Our patient's prodromal symptoms suggest preceding systemic infection, a feature previously noted in APMPPE2. Following the onset of APMPPE, administration of systemic steroid from days 4 to 11 may have promoted resolution of pigmentary epitheliopathy; however, it may also have contributed to the manifestation of adenoviral infection in a further site, that is, keratoconjunctivitis.

To date there is no histology available on an eye with active APMPPE and the aetiology remains a subject of debate. APMPPE can follow systemic triggers such as bacterial3 or viral1,4 infection, vaccination,5 and use of antimicrobial agents,6 suggesting an immune-mediated mechanism. An immunogenetic predisposition for developing APMPPE is supported by the association of HLA types B2 and DR77. Abnormalities of choroidal blood flow, demonstrated by indocyanine green angiography,8 suggest a choroidal vasculitis, and there are reports of associated systemic vasculitides.9,10

Gass11 originally described APMPPE as a benign disease with a good visual prognosis. More recently, it has been suggested that lack of visual recovery may be predicted by the presence of atypical features including age over 60 years, recurrent disease and unilateral involvement.12 In contrast to this, our 36-year-old patient had profoundly different visual outcomes in each eye following new, bilateral disease. This case illustrates that the prognosis in APMPPE with foveal involvement should be guarded.