Acute posterior multifocal placoid pigment epitheliopathy associated with Wegener's granulomatosis.
OBJECTIVE
To report acute posterior multifocal placoid pigment epitheliopathy (APMPPE) in the course of systemic Wegener's granulomatosis.


METHODS
A complete ophthalmologic evaluation was undertaken, including fluorescein and indocyanine green angiography.


RESULTS
Two patients diagnosed with Wegener's granulomatosis developed multiple white-yellow subretinal areas with smooth margins typical of APMPPE. Case 1 presented with bilateral choroidal involvement and a granulomatous anterior uveitis. In Case 2, placoid lesions were diagnosed 2 months after onset of systemic Wegener's granulomatosis. In both cases, fluorescein angiography showed an early hypofluorescence and a late staining of the lesions. Indocyanine green angiography revealed early and late hypofluorescence corresponding to the areas seen clinically. Wegener's granulomatosis was diagnosed histologically in both cases. After combined steroid and cyclophosphamide therapy, the lesions healed, and after a follow-up period of 1.5 and 4 years, respectively, funduscopic and angiographic examinations showed cicatricial lesions.


CONCLUSIONS
Acute posterior multifocal placoid pigment epitheliopathy is a rare manifestation of Wegener's granulomatosis and should be considered a possible systemic involvement of the disease. A prompt, thorough investigation should be undertaken for an underlying systemic disorder.
Chiquet
C
C
Department of Ophthalmology, Edouard Herriot Hospital, Lyon, France.
Lumbroso
L
L
Denis
P
P
Papo
T
T
Durieu
I
I
Lehoang
P
P
D000208:Acute Disease; D000328:Adult; D003520:Cyclophosphamide / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005451:Fluorescein Angiography; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007208:Indocyanine Green; D008297:Male; D008875:Middle Aged; D010857:Pigment Epithelium of Eye / Q000473:pathology*; D012164:Retinal Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D007208:Indocyanine Green