Sections

Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)

Sections Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)
Overview
Presentation
- History
- Physical
- Causes
- Complications
- Show All
DDx
Workup
Treatment
Medication
Media Gallery
References

Treatment

Approach Considerations

Conservative management is recommended; treat ocular complications or systemic vasculitis as indicated.

Medical Care

The treatment of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is controversial. The fundus lesions appear to run a relatively self-limited course with no treatment. Systemic steroids or biologic agents such as tumor necrosis factor inhibitors (eg, infliximab) may be considered in cases involving significant visual problems, recurrent debilitating cases, or cases involving systemic problems.^{[20, 4]}

Surgical Care

In cases complicated by choroidal neovascularization, laser photocoagulation and intravitreal injection of antivascular growth factors (eg, bevacizumab, ranibizumab, aflibercept [off-label use]) can be considered.^[20]

Consultations

Consultations may be indicated if the diagnosis is not clear or if a systemic manifestation indicates such a need (an infrequent occurrence).

Neurologist/neurosurgeon - CNS symptoms
Urologist - Urinalysis findings
Dermatologist - Skin findings
Rheumatologist - Serum immunologic abnormalities
Infectious disease specialist

Diet

No dietary restrictions are indicated.

Activity

No limitations of visual or physical activities are indicated unless systemic manifestations impose limitation of physical activities.

Further Outpatient Care

Monitor the clinical course of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) every 1-2 weeks and for onset of systemic complications.

Medication

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Heiferman MJ, Rahmani S, Jampol LM, Nesper PL, Skondra D, Kim LA, et al. ACUTE POSTERIOR MULTIFOCAL PLACOID PIGMENT EPITHELIOPATHY ON OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY. Retina. 2017 Feb 1. [Medline].
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Baxter KR, Opremcak EM. Panretinal acute multifocal placoid pigment epitheliopathy: a novel posterior uveitis syndrome with HLA-A3 and HLA-C7 association. J Ophthalmic Inflamm Infect. 2013 Feb 4. 3(1):29. [Medline]. [Full Text].
Yeh S, Forooghian F, Wong WT, Faia LJ, Cukras C, Lew JC, et al. Fundus autofluorescence imaging of the white dot syndromes. Arch Ophthalmol. 2010 Jan. 128(1):46-56. [Medline]. [Full Text].
Grkovic D, Oros A, Karadžic J, Bedov T, Gvozdenovic L, Jovanovic S. Acute posterior multifocal placoid pigment epitheliopathy-retinal "white dot syndrome". Med Glas (Zenica). 2013 Feb. 10(1):194-6. [Medline].
Burke TR, Chu CJ, Salvatore S, Bailey C, Dick AD, Lee RWJ, et al. Application of OCT-angiography to characterise the evolution of chorioretinal lesions in acute posterior multifocal placoid pigment epitheliopathy. Eye (Lond). 2017 Oct. 31 (10):1399-1408. [Medline]. [Full Text].
Aoyagi R, Hayashi T, Gekka T, Kozaki K, Tsuneoka H. Multifocal electroretinographic evaluation of macular function in acute posterior multifocal placoid pigment epitheliopathy. Doc Ophthalmol. 2013 Jun. 126(3):253-8. [Medline].
Cheung CM, Yeo IY, Koh A. Photoreceptor changes in acute and resolved acute posterior multifocal placoid pigment epitheliopathy documented by spectral-domain optical coherence tomography. Arch Ophthalmol. 2010 May. 128(5):644-6. [Medline].
Goldenberg D, Habot-Wilner Z, Loewenstein A, Goldstein M. Spectral domain optical coherence tomography classification of acute posterior multifocal placoid pigment epitheliopathy. Retina. 2012 Jul. 32 (7):1403-10. [Medline]. [Full Text].
Mrejen S, Gallego-Pinazo R, Wald KJ, Freund KB. Acute posterior multifocal placoid pigment epitheliopathy as a choroidopathy: what we learned from adaptive optics imaging. JAMA Ophthalmol. 2013 Oct. 131 (10):1363-4. [Medline]. [Full Text].
Michael A. Klufas, MD , Nopasak Phasukkijwatana, MD , Nicholas A. Iafe, BA , et al. Optical Coherence Tomography Angiography Reveals Choriocapillaris Flow Reduction in Placoid Chorioretinitis. Ophthalmology Retina. January–February 2017. Volume 1, Issue 1:77–91. [Full Text].
Hirooka K, Saito W, Saito M, Hashimoto Y, Mori S, Noda K, et al. Increased choroidal blood flow velocity with regression of acute posterior multifocal placoid pigment epitheliopathy. Jpn J Ophthalmol. 2016 May. 60 (3):172-8. [Medline].
Mrejen S, Sarraf D, Chexal S, Wald K, Freund KB. Choroidal Involvement in Acute Posterior Multifocal Placoid Pigment Epitheliopathy. Ophthalmic Surg Lasers Imaging Retina. 2016 Jan. 47 (1):20-6. [Medline].
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Mavrakanas N, Mendrinos E, Tabatabay C, Pournaras CJ. Intravitreal ranibizumab for choroidal neovascularization secondary to acute multifocal posterior placoid pigment epitheliopathy. Acta Ophthalmol. 2010 Mar. 88 (2):e54-5. [Medline].
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O'Halloran HS, Berger JR, Lee WB, Robertson DM, Giovannini JA, Krohel GB, et al. Acute multifocal placoid pigment epitheliopathy and central nervous system involvement: nine new cases and a review of the literature. Ophthalmology. 2001 May. 108(5):861-8. [Medline].
Parmeggiani F, Costagliola C, D'Angelo S, Incorvaia C, Perri P, Sebastiani A. Clear cell renal cell carcinoma associated with bilateral atypical acute posterior multifocal placoid pigment epitheliopathy. Oncology. 2004. 66(6):502-9. [Medline].
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Scheufele TA, Witkin AJ, Schocket LS, Rogers AH, Schuman JS, Ko TH, et al. Photoreceptor atrophy in acute posterior multifocal placoid pigment epitheliopathy demonstrated by optical coherence tomography. Retina. 2005 Dec. 25(8):1109-12. [Medline]. [Full Text].
Smith CH, Savino PJ, Beck RW, Schatz NJ, Sergott RC. Acute posterior multifocal placoid pigment epitheliopathy and cerebral vasculitis. Arch Neurol. 1983 Jan. 40(1):48-50. [Medline].
Thomson SP, Roxburgh ST. Acute posterior multifocal placoid pigment epitheliopathy associated with adenovirus infection. Eye. 2003 May. 17(4):542-4. [Medline].
Uthman I, Najjar DM, Kanj SS, Bashshur Z. Anticardiolipin antibodies in acute multifocal posterior placoid pigment epitheliopathy. Ann Rheum Dis. 2003 Jul. 62(7):687-8. [Medline].
Wolf MD, Folk JC, Panknen CA, Goeken NE. HLA-B7 and HLA-DR2 antigens and acute posterior multifocal placoid pigment epitheliopathy. Arch Ophthalmol. 1990 May. 108(5):698-700. [Medline].
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Media Gallery

Posterior pole of right eye. Early acute posterior multifocal placoid pigment epitheliopathy lesion shows yellowish-white placoid lesion involving the macula and an area just inferior temporal to the macula.
Fluorescein angiography showing peripheral hypofluorescence and central leakage of the lesion inferior temporal to the macula.
Posterior pole of left eye of same patient showing acute posterior pole placoid lesion.
Fluorescein angiogram of the patient above in late phase showing late staining of placoid areas.
Inferior nasal of right eye of the same patient approximately 2 months later, showing scattered areas of retinal pigment epithelium atrophy and hyperplasia.
Fluorescein angiography of same patient in late phase showing areas of late staining.
This image shows localized scleritis superiorly.

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Author

Lakshmana M Kooragayala, MD Vitreo-retinal Surgeon, Marietta Eye Clinic

Lakshmana M Kooragayala, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

R Christopher Walton, MD Adjunct Professor, Department of Ophthalmology, University of Texas Health Science Center at San Antonio

R Christopher Walton, MD is a member of the following medical societies: American Academy of Ophthalmology, American Uveitis Society

Disclosure: Nothing to disclose.

Chief Editor

C Stephen Foster, MD, FACS, FACR, FAAO, FARVO Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institution

C Stephen Foster, MD, FACS, FACR, FAAO, FARVO is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Association of Immunologists, American College of Rheumatology, American College of Surgeons, American Federation for Clinical Research, American Medical Association, American Society for Microbiology, American Uveitis Society, Association for Research in Vision and Ophthalmology, Massachusetts Medical Society, Royal Society of Medicine, Sigma Xi

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Aldeyra Therapeutics (Lexington, MA); Bausch & Lomb Surgical, Inc (Rancho Cucamonga, CA); Eyegate Pharma (Waltham, MA); Novartis (Cambridge, MA); pSivida (Watertown, MA); Xoma (Berkeley, CA); Allakos (Redwood City, CA)<br/>Serve(d) as a speaker or a member of a speakers bureau for: Alcon (Geneva, Switzerland); Allergan (Dublin, Ireland); Mallinckrodt (Staines-upon-Thames, United Kingdom)<br/>Received research grant from: Alcon; Aldeyra Therapeutics; Allakos Pharmaceuticals; Allergan; Bausch & Lomb; Clearside Biomedical; Dompé pharmaceutical; Eyegate Pharma; Mallinckrodt pharmaceuticals; Novartis; pSivida; Santen; Aciont.

Additional Contributors

Andrew A Dahl, MD, FACS Assistant Professor of Surgery (Ophthalmology), New York College of Medicine (NYCOM); Director of Residency Ophthalmology Training, The Institute for Family Health and Mid-Hudson Family Practice Residency Program; Staff Ophthalmologist, Telluride Medical Center

Andrew A Dahl, MD, FACS is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, American Intraocular Lens Society, American Medical Association, American Society of Cataract and Refractive Surgery, Contact Lens Association of Ophthalmologists, Medical Society of the State of New York, New York State Ophthalmological Society, Outpatient Ophthalmic Surgery Society

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous coauthor, James P Ganley, MD, PharmD, DrPH, to the development and writing of this article.

Sections Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)
Overview
Presentation
- History
- Physical
- Causes
- Complications
- Show All
DDx
Workup
Treatment
Medication
Media Gallery
References

Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) Treatment & Management

Approach Considerations

Medical Care

Surgical Care

Consultations

Diet

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