Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) Clinical Presentation

Updated: Jul 22, 2019
  • Author: Lakshmana M Kooragayala, MD; Chief Editor: C Stephen Foster, MD, FACS, FACR, FAAO, FARVO  more...
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Presentation

History

Prodromal symptoms of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) may include the following:

  • Prior viral or flulike syndrome - Occurs in approximately one third of patients; symptoms include fever, cough, swollen lymph glands, nausea, vomiting, myalgia, malaise, muscle and joint tenderness
  • Moderate-to-severe headache
  • Neurologic symptoms (rare) - Transient aphasia, numbness and weakness of extremities, feelings of clumsiness

Early-stage symptoms may include the following:

  • Acute decrease in visual acuity
  • Blotchy scotomata
  • Photopsia
  • Metamorphopsia/micropsia
  • Photophobia
  • Conjunctival injection/episcleritis (rare)

Late-stage symptoms may include the following:

  • Mild visual impairment (20/25 to 20/40), common
  • Significant visual loss (20/200), rare
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Physical

Patients should have a complete eye examination, including visual acuity, pupillary reactions, slit lamp examination, and dilated indirect ophthalmoscopy.

Early stage

Visual acuity may be normal; if the macula is involved, vision can decrease to 20/200 within days after onset.

Extraocular muscle function is normal unless cerebral vasculitis develops.

No relative afferent pupillary defect is present.

Conjunctivitis or episcleritis is rarely present.

If present, anterior chamber reaction usually is mild, although fibrinous inflammation has been noted.

Vitreous cells may be found in up to 50% of eyes that are affected, but it is usually mild if present.

Retinal findings

Retinal findings are the main feature of the disease.

Multiple subretinal placoid yellow-white lesions are seen in both eyes. In some cases, the lesions are unilateral with involvement of the second eye either within a short period of time or after an extended period. New lesions may occur in the affected eye as old lesions begin their resolution.

Rarely, a well-demarcated serous retinal detachment may develop in the posterior retina and rarely located anterior to the equator.

Optic nerve involvement

Blurring of disc margins, hyperemia, edema, and superficial hemorrhages may be found.

Papillitis and optic neuritis are a less frequent occurrence.

Venous compression within the optic nerve may lead to retinal vein distension and, rarely, to central retinal vein occlusion.

Late stage

Individual placoid lesions resolve over several weeks as a natural course, along with other signs of inflammation.

Resolution of the placoid lesions is characterized by well-demarcated areas of retinal pigment epithelial loss with presence of diffuse fine foci of hyperplasia, along with similar choroidal findings.

Long-term retinal pigment epithelium (RPE) changes may continue to develop long after recovery.

In most instances, the visual acuity returns to 20/30 or better; initial visual recovery is rapid, but final recovery may take up to 6 months.

Recurrences

APMPPE tends to occur bilaterally, although both eyes may not be affected at the same time.

Long-term follow-up studies suggest that recurrences may develop in up to 50% of patients.

Systemic manifestations

Systemic manifestations, believed to result from diffuse multisystem vasculitis, may include the following:

  • Erythema nodosum

  • Thyroiditis

  • Microvascular nephropathy

  • Neurologic manifestations include cerebral vasculitis, transient ischemic attacks, fixed neurologic deficits, transient or permanent low-tone hearing loss, vertigo, labyrinthitis, and meningoencephalitis.

Rare ocular manifestations

These may include the following:

  • Perilimbal corneal stromal infiltrates

  • Serous retinal detachment

  • Retinal periphlebitis

  • Retinal venous dilation and tortuosity

  • Ciliary flush

  • Corneal edema

  • Limbal corneal neovascularization

  • Limbal corneal thinning

  • Anterior and posterior synechiae

  • Koeppe nodules

  • Relative afferent pupillary defect

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Causes

APMPPE has been known to occur after different conditions or in conjunction with a broad variety of disorders, as follows:

  • Tuberculosis exposure

  • Hepatitis B vaccination

  • Group A streptococcal infection

  • Swine flu vaccination

  • Tuberculin skin testing

  • Penicillin therapy

  • Erythromycin therapy

  • Adenovirus 5 infection

  • Use of oral contraceptives

  • Use of hormone replacements

  • Clear cell renal cell carcinoma

  • Systemic necrotizing vasculitis

  • Meningococcal C conjugate vaccine [5]

  • Adenovirus infection

  • Influenza vaccination [6]

  • Varicella vaccination [7]

The actual cause of APMPPE is not known. Approximately one third of patients in some series have a history of recent viral illness with a flulike syndrome, upper respiratory infection, or other systemic illness several days to a few weeks before appearance of the ocular lesions. Fever, headache, malaise, gastrointestinal, or upper respiratory symptoms may be preceding symptoms.

Since APMPPE seems to occur after diverse infectious diseases and other stimuli, many investigators believe that it is likely an immune disorder. A higher frequency of human leukocyte antigen B7 (HLA-B7) and human leukocyte antigen DR2 (HLA-DR2) have been reported, suggesting an inherited tendency for the disorder, and that several infectious agents and or other triggers may be the stimulus of APMPPE in susceptible individuals. [8] The general consensus is that the systemic and ocular causes of APMPPE may be a generalized vasculitis leading to choroidal lobular nonperfusion.

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Complications

Choroidal neovascularization can develop in areas of previously healed lesions associated with bleeding and/or subretinal fluid. If the lesion is close to or under the foveal system, patients can experience sudden vision decrease.

See the list below:

  • Subretinal neovascularization

  • Retinal vein occlusion

  • Death or other permanent neurologic sequelae from cerebral vasculitis; may include muscle paralysis and permanent hearing loss

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