Orange pigment sediment overlying small choroidal melanoma.

Comment. To our knowledge, only 2 other studies have reported death secondary to cerebrovascular complications of APMPPE. In our case, like the 2 other reports, death occurred when oral prednisone was rapidly tapered to a dose of 20 mg/d. From a review of 9 patients with APMPPE who had strokes, patients were less likely to have a second stroke or die if they received high-dose corticosteroids (60 mg prednisone equivalent or greater) followed by a prolonged, gradual taper or initiation of a steroid-sparing agent. The choroidal inflammatory lesions of APMPPE have been associated with systemic necrotizing vasculitides, such as Wegener granulomatosis and polyarteritis nodosa, that require aggressive, prolonged immune suppression to prevent significant morbidity or death. Both angiographic and histopathologic evidence of granulomatous cerebral vasculitis has been demonstrated in patients with cerebrovascular complications from APMPPE. We recommend a low threshold for neurologic and rheumatologic consultation and initiation of high-dose corticosteroids. Once steroids are initiated, they should not be tapered until quiescence of choroidal inflammation and resolution of the neurologic symptoms, as these may represent active vasculitic disease. Thereafter, we recommend tapering corticosteroids according to expert guidelines proposed for other ocular inflammatory disorders. In these rare circumstances, a cautious corticosteroid taper with close monitoring of systemic adverse effects may avoid cerebrovascular complications and death.