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Acute exudative polymorphous vitelliform maculopathy syndrome.

Clement K Chan1, J Donald M Gass, Steven G Lin

  • 1Southern California Desert Retina Consultants, PO Box 2467, Palm Springs, CA 92263, USA. PSChan@aol.com

Retina (Philadelphia, Pa.)
|September 16, 2003
PubMed
Summary
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Polymorphous maculopathy syndrome (PMS) is an unusual tapetoretinal disorder. This case report details its clinical characteristics, including retinal detachment, and suggests a possible viral cause.

Area of Science:

  • Ophthalmology
  • Retinal Diseases
  • Macular Disorders

Background:

  • Polymorphous maculopathy syndrome (PMS) is a rare tapetoretinal disorder initially described by Gass.
  • Understanding the clinical spectrum and potential etiologies of PMS is crucial for diagnosis and management.

Observation:

  • A female patient presented with characteristic yellowish lesions in a honeycomb pattern at the retinal pigment epithelium (RPE) level.
  • Optical coherence tomography (OCT) revealed a shallow macular detachment and perifoveolar yellow rings.
  • Subretinal yellowish deposits evolved over time, and a rhegmatogenous retinal detachment occurred in one eye.

Findings:

  • Electrophysiology demonstrated reduced electroretinogram and electrooculogram amplitudes and abnormal dark adaptometry.

Related Experiment Videos

  • Fluorescein angiography showed early hyperfluorescence and late staining of lesions and perifoveolar rings.
  • Genetic analysis excluded mutations in the bestrophin and peripherin/RDS genes, while serology suggested a possible coxsackie virus infection.

    • Implications:

    • This report expands the known clinical features of PMS, including its occurrence in women and association with retinal detachment.
    • PMS shares similarities with Harada disease and Best disease but has distinct features.
    • Further research is needed to elucidate the relationship between PMS, viral infections, and other genetic factors.