Acute posterior multifocal placoid pigment epitheliopathy with cerebral vasculitis: a multisystem granulomatous disease.

operatively, we suspected that this most likely represented a benign smooth muscle tumor that had eroded through the iris root. Amyloid can occur as a localized ocular or widespread systemic process. Approximately 4% of amyloid deposits in the head and neck region involve the orbit. Amyloid deposits in ophthalmic structures can occur as a primary or secondary process. Primary deposits (which can be familial or sporadic) occur in the absence of an associated disease. Secondary deposits have been noted after a myriad of processes, including trauma, infection,myeloproliferativedisorders, and immune-mediated diseases. Most of the reported ophthalmic cases have been in association with familial amyloidosis with systemic involvement. Some cases have been noted to have only ophthalmic deposition of amyloid without evidence of systemic disease. Amyloid deposits in association with myeloproliferative entities suchas lymphomasorplasmacellproliferations can produce paraproteinemias and involve the eye. In our case, the negative study results make this entity unlikely at present. In patients with extraosseous plasmacytoma, a myeloma develops within 10 years in 10% to 30% compared with 55% in patientswithosseousplasmacytomas. Amyloid deposits can also occur in association with rheumatologic diseases, although involvement with systemic lupus erythematosus is distinctly uncommon. Our patient has either a primary amyloid deposit from an extraosseous plasmacytoma or a focal iris–ciliary amyloid deposit in association with systemic lupus erythematous. It is conceivable that benign but aberrantly localized plasma cells are part of the systemic lupus erythematosus process in this patient. Alternatively, there is an increase in lymphomas in patients with systemic lupus erythematosus, even when they are not treated with immunosuppression. Amyloid in association with systemic lupus erythematosus is quite rare and usually manifests as renal involvement. Fewer than 20 cases have been reported. This case illustrates the problem, despite newer diagnostic techniques, of potential diagnostic errors in anterior uveal tumor diagnosis. Our patient is probably at risk for local and systemic recurrence and is being closely observed.