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Ocular Immunology and Inflammation Volume 30, 2022 - Issue 2
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Research Article

Clinical Correlation between Acute Exudative Polymorphous Paraneoplastic Vitelliform Maculopathy and Metastatic Melanoma Disease Activity: A 48-month Longitudinal Case Report

Claire M Muellera Department of Ophthalmology, Oregon Health & Science University Casey Eye Institute, Portland, OR, USAORCID Iconhttps://orcid.org/0000-0002-9415-1744View further author information
, MDORCID Icon,
Sara L Hojjatieb Department of Hematology and Oncology, Emory University School of Medicine, Atlanta, GA, USAView further author information
, MD,
David H Lawsonc Emory University School of Medicine, Professor of Hematology and Medical Oncology, Winship Cancer Institute of Emory University, Atlanta, GA, USAView further author information
, MD,
Nieraj Jaind Emory University School of Medicine, Assistant Professor of Ophthalmology, Section of Vitreoretinal Surgery and Diseases, Atlanta, GA, USAView further author information
, MD,
Joshua Robinsone Palmetto Retina Center, West Columbia, SC, USAView further author information
, MD,
Mohammad K Khanf Emory University School of Medicine, Associate Professor of Radiation Oncology, Winship Cancer Institute of Emory University, Atlanta, GA, USAView further author information
, MD, Ph.D,
Melinda L Yushakg Emory University School of Medicine, Assistant Professor of Hematology and Medical Oncology, Winship Cancer Institute of Emory University, Atlanta, GA, USAView further author information
, MD &
Ghazala A Datoo O’Keefeh Emory University School of Medicine, Assistant Professor of Ophthalmology, Section of Vitreoretinal Surgery and Diseases, Section of Uveitis and Vasculitis, Atlanta, GA, USACorrespondence[email protected]
View further author information
, MD show all
Pages 330-337 | Received 14 May 2020, Accepted 19 Aug 2020, Published online: 06 Oct 2020
 
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ABSTRACT

Purpose

Longitudinal evaluation of acute exudative polymorphous paraneoplastic vitelliform maculopathy (AEPPVM) following diagnosis and treatment of metastatic melanoma.

Methods

Case report of a 47-year-old male with unknown primary metastatic melanoma and AEPPVM monitored before and during melanoma treatment using clinical exam, retinal imaging, and electroretinograms (ERG). Genetic testing and autoantibody panels were performed.

Results

He presented within a month of metastatic melanoma diagnosis with numerous bilateral vitelliform lesions in the posterior pole, consistent with AEPPVM. Metastatic disease was treated with immunotherapy, radiosurgery, and radiation over 48 months. Maculopathy and metastatic disease improved and worsened in parallel. Genetic testing was negative for bestrophin-1. An autoantibody panel was positive for anti-recoverin and transducin-α.

Conclusion

AEPPVM is an uncommon paraneoplastic retinopathy found in patients with metastatic malignancy. To our knowledge, this is the first report demonstrating a temporal association between metastatic disease activity and quantifiable changes in retinal imaging over a 4-year period.

Declaration of Interest

Claire M. Mueller: None

Sara Hojjatie: None

David H. Lawson: None

Nieraj Jain: Funded by Foundation Fighting Blindness CD-C-0918-0748-EEC

Joshua Robinson: None

Mohammad Khan: None

Melinda Yushak: Winship Cancer Institute Start Up Funds (source of funding for genetic testing)

Ghazala A. D. O’Keefe: Eyepoint pharmaceuticals, advisory board.

Additional information

Funding

This work was supported by the Foundation Fighting Blindness [CD-C-0918-0748-EEC]; Winship Cancer Institute Start Up Funds.

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