Abstract Details

APMPPE is a rare ‘white dot syndrome’ which causes visual abnormalities such as photopsia and blotchy scotoma, proceeded by flu like symptoms such as fever, myalgias and headache in about one third of patients. Direct fundoscopy classically shows multiple flat, yellow white lesions at the level of the retinal pigment epithelium in the posterior pole. The majority of cases are idiopathic and self-limiting, though occasionally it can result in aseptic meningitis and CNS vasculitis.

We present a case of Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) as the presenting feature of systemic sarcoidosis.

A 44-year-old gentleman presented with a one-week history of gradual loss of vision in both eyes, preceded by 10 days of low-grade fevers, arthralgias and headache. He described flashing lights in his vision, ‘like flames of a fire.’ Ocular examination and ancillary multimodal imaging were consistent with APMPPE.  

ESR was elevated at 65 mm/hr. CT Intracranial Angiogram and MRI Brain with contrast were normal. CSF showed pleocytosis with 141 lymphocytes per cmm, CSF protein was elevated at 0.71 g/L with normal glucose of 3.9 mmol/L. Extensive infectious disease screening, including viral PCR, HIV, syphilis, and TB cultures were negative.  US temporal arteries revealed hyperechoic thickening, but temporal artery biopsy was normal. CT TAP revealed hilar lymphadenopathy. A EBUS was performed which showed non-necrotising granulomatous change consistent with sarcoidosis. He was started on high dose oral prednisolone on admission with an improvement in his vision and headache.

APMPPE is a rare cause of visual loss in young patients preceded by photopsia and often associated with systemic symptoms. All patients presenting with same should have extensive work up for possible associated systemic causes such as sarcoidosis, as well as exclusion of potential co-existing CNS vasculitis. Patients typically respond well to steroids.