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Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) is an acquired inflammatory uveitis that belongs to the heterogenous group of white dot syndromes in which light-coloured (yellowish-white )lesions begin to form in the macular area of the retina.Early in the course of the disease, the lesions cause acute and marked vision loss (if it interferes with the optic nerve) that ranges from mild to severe but usually transient in nature. Classified as an inflammatory disorder that is usually bilateral and acute in onset but self-limiting. The lesions leave behind some pigmentation, but visual acuity eventually improves even without any treatment (providing scaring doesn't interfering with optic nerve).

It occurs more commonly in females, and is more likely to affect a persons between 20–30 years of ages but has been seen in people 16 to 40 years of age . It is known to occur after or concurrently with a systemic infection (but not always), showing that it is related generally to an altered immune system. Recurrent episodes can happen, but is extremely rare.^[1]^[2]

Etiology

The aetiology of the inflammation remains unknown, with various theories of it occurring as an autoimmune response to a mild infection, or the possibility of it being viral because of the preceding flu-like illness that generally accompanies it.^[1]^[3] It is usually associated with HLA-B7 and HLA-DR2

Presentation

The onset of ocular symptoms are usually preceded by episode of viral or flu-like symptoms such as fever, cough or sore throat (however this is not always the case). Patients can typically present erithema nadosum, livido reticularus, bilateral uveitis, and sudden onset of marked visual loss associated with the appearance of multiple lesions in the retina. These lesions may be colored from grey-white to cream-shaded yellow. Other symptoms include scotomata and photopsia. In weeks to a month times the lesions begin to clear and disappear (with prednisone) leaving behind areas of retinal pigment epithelial atrophy and diffuse fine pigmentation (scaring) . Rarely choroidal neovascularization occur as a late onset complication.^[3]

Diagnosis

Diagnosis is made by an ophthalmologist during eye examination. Further tests such as Fluorescein angiography and/or lumbar puncture is usually performed to confirm the diagnosis. Similar auto immune disorder that can be confused with APMPPE, is neuro sarcoidosis.

Treatment

Due to self-limited nature of the disease treatment is generally not required. In the case where lesions appear to be interfering or boarding the optic nerve, methyl prednisone is prescribed.

Prognosis

Vision does improve in almost all cases. Rarely a patient may suffer permanent visual loss associated with lesion on optic nerve.

Rarely, coexisting vasculitis may cause neurological complications. These occurrences can start with mild headaches that steadily increase in pain and threshold and include attacks of dysesthesia. It has been found that this type of deterioration happens usually if the lesions involve the fovea. This is not always the case. I presented with no headache, just fell, and had right hand side weakness, upon presentation to hospital still no head ache but also had slurred speech. This followed another cva (cerebral vascular accident) with reoccurring Tia's. The risk associated with have cva associated with APMPPE is extremely rare.^[1]^[4]

References

^ ^a ^b ^c Comu, S; Verstraeten, T; Rinkoff, JS; Busis, NA (May 1996). "Neurological manifestations of acute posterior multifocal placoid pigment epitheliopathy.". Stroke; a journal of cerebral circulation 27 (5): 996–1001. doi:10.1161/01.str.27.5.996. PMID 8623125.
^ Jones, N.P. (1995). Acute posterior multifocal placoid pigment epitheliopathy. group.bmj.com. pp. 1–oooooo7. Retrieved 2009-09-15.
^ ^a ^b De Vries, J.J. "Acute Posterior Multifocal Placoid Pigment Epitheliopathy With Cerebral Vasculitis: A Multisystem Granulomatous Disease - Archives of Ophthalmology". http://archopht.ama-assn.org/. Retrieved 2009-09-15.
^ Wolf, Mitchell. "Long-term Visual Function in Acute Posterior Multifocal Placoid Pigment Epitheliopathy - Archives of Ophthalmology". http://archopht.ama-assn.org/. Retrieved 2009-09-15.

English Journal

The role of fundus autofluorescence imaging in the study of the course of posterior uveitis disorders.

Malamos P1, Masaoutis P2, Georgalas I2, Maselos S3, Andrianopoulos K2, Koutsandrea C2, Markomichelakis NN3.
BioMed research international.Biomed Res Int.2015;2015:247469. doi: 10.1155/2015/247469. Epub 2015 Jan 28.
BACKGROUND: To evaluate the correlation of fundus autofluorescence (FAF) with indocyanine green angiography (ICGA) in patients with various posterior uveitis disorders.METHODS: Interventional case series including 23 eyes of 15 patients with diagnosis of a specific type of retinochoroiditis, such as
PMID 25695054

[Acute posterior multifocal placoid pigment epitheliopathy - case report].

Stepanov A, Feuermannová A, Studnička J, Hejsek L, Burova M, Jirásková N, Rozsíval P.
Ceská a slovenská oftalmologie : casopis Ceské oftalmologické spolecnosti a Slovenské oftalmologické spolecnosti.Cesk Slov Oftalmol.2014 Apr;70(2):72-6.
Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) affects individuals between 20 to 30 years of age and ocular manifestations are often preceded by flu-like illness. Symptoms of the disease include acute vision decrease associated with central and paracentral scotoma. Impairment of
PMID 25030317

Functional and anatomic changes in acute multifocal placoid pigment epitheliopathy: a case report.

Cennamo G1, Nicoletti G, Amoroso F, Velotti N, Soda M, De Crecchio G.
European journal of ophthalmology.Eur J Ophthalmol.2014 Mar-Apr;24(2):286-9. doi: 10.5301/ejo.5000374. Epub 2013 Sep 23.
PURPOSE: To report the functional and anatomic features of a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE).METHODS: Case report.RESULTS: A 30-year-old man presented with a decrease of visual acuity that started a few days earlier. Fundus examination showed multiple, subr
PMID 24170516

Japanese Journal

急性後部多発性斑状色素上皮症に前部虚血性視神経を合併した一例

泉谷瑶子,戸成匡宏,栗本拓治,菅澤淳,池田恒彦
神経眼科 31(2), 214-219, 2014
… 急性後部多発性斑状色素上皮症（APMPPE）に前部虚血性視神経症（AION）を合併した一例を報告する．症例は75歳女性，左眼の下方視野欠損を自覚し受診．視力は右眼（1.2），左眼（0.4）．左眼視神経乳頭は発赤腫脹し，後極部に灰白色の斑状滲出班を多数認めた．FAでは滲出班に一致して，造影の逆転現象が認められた．IAでは左眼の滲出班部は一貫して低蛍光を示した．ゴールドマン動的視野検査（GP）では，下水平半盲 …
NAID 130004699043

眼底周辺部に滲出斑が多発した急性後部多発性斑状色素上皮症の1例

池辺徹,武富練一郎,木許賢一
眼科臨床紀要 6(7), 580-584, 2013
NAID 40019742959

急性網膜色素上皮炎様所見を合併した急性後部多発性斑状色素上皮症の1例 (特集第63回日本臨床眼科学会講演集(3))

木嶋理紀,齋藤航,藤谷顕雄 [他]
臨床眼科 64(5), 707-711, 2010-05
NAID 40017157512

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