Acute posterior multifocal placoid pigment epitheliopathy associated with a systemic necrotizing vasculitis.
OBJECTIVE
The authors present a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) associated with a systemic necrotizing vasculitis with mixed features of Wegener granulomatosis and polyarteritis nodosa (PAN).


METHODS
Case report.


RESULTS
A 29-year-old woman developed a severe nosebleed, followed by a low-grade fever, night sweats, and a productive cough. One month later, she began experiencing high fevers, headache, myalgia, neck stiffness, and abdominal pain as well as bilateral blurred vision from APMPPE. Systemic evaluation revealed nasal ulcerations, bilateral pleural effusions, and a bilateral maxillary and ethmoid sinusitis, consistent with Wegener granulomatosis. However, ANCA testing was negative, and a renal and mesenteric angiogram showed aneurysmal dilatations suggestive of PAN. Her ocular and systemic symptoms markedly improved with systemic corticosteroids.


CONCLUSIONS
The cause of APMPPE is unknown. This case of APMPPE associated with systemic necrotizing vasculitis provides support for the choroid as being primarily involved by a diffuse vasculitic process that interrupts choroidal perfusion and causes the characteristic fundus findings in APMPPE.
Hsu
Cynthia T
CT
Wilmer Eye Institute, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Harlan
Joseph B
JB
Goldberg
Morton F
MF
Dunn
James P
JP
D000328:Adult; D004359:Drug Therapy, Combination; D015521:Ethmoid Sinusitis / Q000150:complications; D005260:Female; D005451:Fluorescein Angiography; D005938:Glucocorticoids / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis; D006801:Humans; D015523:Maxillary Sinusitis / Q000150:complications; D008776:Methylprednisolone Hemisuccinate / Q000627:therapeutic use; D010857:Pigment Epithelium of Eye / Q000473:pathology*; D010488:Polyarteritis Nodosa / Q000175:diagnosis; D011239:Prednisolone / Q000627:therapeutic use; D015882:Retinal Necrosis Syndrome, Acute / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D031300:Retinal Vasculitis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy
D005938:Glucocorticoids; D008776:Methylprednisolone Hemisuccinate; D011239:Prednisolone