Unilateral Acute Multifocal Posterior Placoid Pigment Epitheliopathy with Papillitis

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Introduction: Gas in 1968 described Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) as being a condition that caused acute and rapid loss of central vision due to multiple pale lesions at the level of Retinal pigment epithelium (RPE) in the posterior pole. (1) It is a self-limiting condition which recovers spontaneously over a three weeks period leaving residual pigment epithelial alterations.(2) The typical features in acute phase include cream colored placoid lesions at the level of RPE, early hypofluorescence and late hyperfluorescence of the lesions on Fundus fluorescein angiography (FFA).(2) It is usually followed by a viral flu like prodrome in 1/3 of the patients. Neurological manifestations of APMPPE include Headaches being commonest while others are paraesthesias, vertigo, psychosis and more severe complications, Cerebrospinal fluid pleocytosis stroke and cerebral vasculitis.(3, 4) Association of APMPPE with systemic Vasculitis in the patients with positive perinuclear antineutrophil cytoplasmic antibody has been reported.(5, 6) Other systemic inflammatory diseases suggesting an underlying immune mediated or an inflammatory mechanism include erythema nodosum,(7-9) juvenile rheumatoid arthritis,(10) thyroiditis,(11, 12) nephritis,(5, 6, 13) ulcerative colitis(14) and Adenoviral infections(15) . Many granulomatous diseases have documented associations with APMPPE, including Wegener’s granulomatosis,(16-18) Pulmonary tuberculosis(19) and Sarcoidosis. The widespread involvement of Retinal Pigment Epithelium (RPE), flat (placoid) nature of the lesions and absence of overlying serous retinal detachment and minimal choroidal involvement lead Gass to conclude RPE was primary focus of inflammation.(1) ... ... middle of paper ... ...th recurrent stroke. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society. 2009;29(2):111-8. Epub 2009/06/06. 30. Bures-Jelstrup A, Adan A, Casaroli-Marano R. [Acute posterior multifocal placoid pigment epitheliopathy. Study of 16 cases]. Archivos de la Sociedad Espanola de Oftalmologia. 2007;82(5):291-7. Epub 2007/05/23. Epiteliopatia pigmentaria placoide posterior multifocal aguda. Estudio de 16 casos. 31. Celis Sanchez J, Gonzalez Del Valle F, Alonso Martinez I, Romero Royo C, Lopez Ferrando N, Zarco Tejada JM. [Bilateral serous retinal detachments in acute posterior multifocal placoid pigmentous epitheliopathy]. Archivos de la Sociedad Espanola de Oftalmologia. 2001;76(11):679-82. Epub 2001/11/21. Desprendimientos retinianos serosos bilaterales en la epiteliopatia pigmentaria placoide multifocal posterior aguda.

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