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Figure 1. This fundus exam of the patient’s right eye reveals multiple creamy white lesions perifoveally.
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Figure 2. Lesions similar to the ones in the patient’s right eye were found in the fundus exam of his left eye as well.
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Figure 3. This SD-OCT scan through a lesion of the right (top) and left (bottom) eye shows interruption of the photoreceptor inner and outer segment layers.
A 22-year-old man with a medical history significant for migraine headaches with visual aura, asthma, and concussion presented to the emergency department with 1 week of visual disturbances in both eyes. He described seeing persistent purple lights in both eyes that do not move. He has had a headache for the past several weeks and was dizzy on the day of presentation.
On exam, visual acuity was 20/20 in both eyes, intraocular pressures (IOP) were 11 OD and 13 OS, with no relative afferent pupillary defect. Confrontational visual fields were full, and the anterior segment slit lamp exam was unremarkable. Fundus examination showed bilateral perifoveal creamy white lesions (Figure 1 and 2). Spectral domain optical coherence tomography (SD-OCT) of the macula shows disruption of the inner segment (IS) and outer segment (OS) of the fovea at the sites of the lesions (Figure 3).
The presence of bilateral perifoveal white dots in a young man with corresponding IS and OS disruption on SD-OCT was diagnostic for acute posterior multifocal placoid pigment epitheliopathy (APMPPE). APMPPE is a rare inflammatory disease, classically considered part of the...
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The presence of bilateral perifoveal white dots in a young man with corresponding IS and OS disruption on SD-OCT was diagnostic for acute posterior multifocal placoid pigment epitheliopathy (APMPPE).
APMPPE is a rare inflammatory disease, classically considered part of the white dot syndromes.1 Patients typically present with bilateral sudden painless vision loss, photopsias, and paracentral scotomas1 and can have a flu-like prodrome or headaches. The mean age of presentation is 25 years old and there is no significant sex bias.2
The key criteria for diagnosing APMPPE are placoid creamy yellow or gray-white lesions, early and diffuse hyperfluorescence late on fluorescein angiogram in the area of the lesions, and negative syphilis and sarcoid testing.2
Lesions are typically bilateral (91%), multifocal (67%), larger than 250 µm (92%), placoid in appearance (97%), and located in the posterior pole (96%).2 The disease presentation tends to be acute (83%) in onset and has no other signs of intraocular inflammation (no anterior chamber or vitreous cell or flare, no keratic precipitates, lack of retinal vascular sheathing or leakage).2
It is believed that APMPPE results from inflammation occurring primarily in the choriocapillaris with secondary disruption of the overlying photoreceptors.3 This hypothesis is supported by multimodal imaging including indocyanine green angiography, which shows hypofluorescence of the choroidal vasculature at the sites of the lesion, OCT angiography (OCT-A), which shows lack of flow signal in the choriocapillaris, and SD-OCT, which shows disruption of the outer retina and ellipsoid zone.4-6
APMPPE is associated with infectious and autoimmune conditions including tuberculosis, psoriasis, sarcoidosis, erythema nodosum, eczema, diabetes mellitus, COVID-19, and various vaccines.1 Notably, several human leukocyte antigen (HLA) subtypes increase the risk of disease, including HLA-B7 (relative risk of 3.38) and HLA-DR2 (relative risk of 3.34).7
Patients with APMPPE can develop choroidal neovascularization, but it’s rare.9 They may also have associated neurological complications including cerebral vasculitis, headache, cranial nerve 6 palsy, transient hearing loss, meningoencephalitis, cavernous sinus thrombosis, viral meningitis and peripheral neuropathy.10
Brain magnetic resonance imaging (MRI) is indicated for patients with headache or neurologic symptoms and brain MR angiography if cerebral vasculitis is suspected, both of which were performed for our patient and were normal.
Cases of APMPPE typically self-resolve in 4 to 8 weeks. While roughly 50% of APMPPE patients regain 20/20 vision, approximately 60% have residual visual symptoms of some kind, especially if there is foveal involvement.8 There is a lack of data regarding the efficacy of treatment for this rare disease, though steroids have been used for acute presentations while antimetabolites such as mycophenolate mofetil have been used for recurrent disease.1,8 Patients with focal neurological symptoms are treated with intravenous methylprednisolone.1 Notably, recurrent disease may indicate a diagnosis of relentless placoid chorioretinitis, which has a more severe course with high rate of recurrent sometimes requiring immunomodulatory therapy.
Our patient was managed conservatively and continues to have 20/20 vision in both eyes.
This case was contributed by James Harris, MD, PhD, a resident physician at Massachusetts Eye and Ear and Harvard Medical School, Boston.
The case was edited by Grayson W. Armstrong, MD, MPH, an instructor of ophthalmology at Massachusetts Eye and Ear and Harvard Medical School and Director of Ophthalmology Emergency Service.
Drs Harris and Armstrong wish to thank George Papaliodis, MD, for his expertise with this case.
References
1. Testi I, Vermeirsch S, Pavesio C. Acute posterior multifocal placoid pigment epitheliopathy (APMPPE). J Ophthalmic Inflamm Infect. 2021;11(9):31. doi:10.1186/s12348-021-00263-1
2. Standardization of uveitis nomenclature working group. Classification criteria for acute posterior multifocal placoid pigment epitheliopathy. Am J Ophthalmol 2021;228;174-181. doi:10.1016/j.ajo.2021.03.056
3. Van Buskirk EM, Lessell S, Friedman E. Pigmentary epitheliopathy and erythema nodosum. Arch Ophthalmol. 1971 Mar;85(3):369-72. doi:10.1001/archopht.1971.00990050371025
4. Howe LJ, Graham EM, Fitzke F, Bhandari A, Marshall J. Choroidal hypoperfusion in acute posterior multifocal placoid pigment epitheliopathy. An indocyanine green angiography study. Ophthalmol. 1995;102(5);790-798. doi:10.1016/s0161-6420(95)30955-4
5. Salvatore S, Steeples LR, Ross AH, et al. Multimodal imaging in acute posterior multifocal placoid pigment epitheliopathy demonstrating obstruction of the choriocapillaris. Ophthalmic Surg Lasers Imaging Retina. 2016;47(7), 677-681. doi:10.3928/23258160-20160707-12
6. Scheufele TA, Witkin AJ, Schocket L, et al. Photoreceptor atrophy in acute posterior multifocal placoid pigment epitheliopathy demonstrated by optical coherence tomography. Retina. 2005;25(7):1109-1112. doi:10.1097/00006982-200512000-00027
7. Wolf MD, Folk JC, Panknen CA, Goeken NE. HLA-B7 and HLA-DR2 antigens and acute posterior multifocal placoid pigment epitheliopathy. Arch Ophthalmol. 1990;108(5):698-700. doi:10.1001/archopht.1990.01070070084040
8. Fiore T, Iaccheri B, Androudi S, et al. Acute posterior multifocal placoid pigment epitheliopathy: outcome and visual prognosis. Retina. 2009;29(7):994-1001. doi:10.1097/IAE.0b013e3181a0bd15
9. Mavrakanas N, Mendrinos E, Tabatabay C, Pournaras CJ. Intravitreal ranibizumab for choroidal neovascularization secondary to acute multifocal posterior placoid pigment epitheliopathy. Acta Ophthalmol. 2010;88(3):e54-55 (2010). doi:10.1111/j.1755-3768.2009.01541.x
10. Algahtani H, Alkhotani A, Shirah B. Neurological manifestations of acute posterior multifocal placoid pigment epitheliopathy. J Clin Neurol. 2016;12(4):460-467. doi:10.3988/jcn.2016.12.4.460
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