Purpose: To present a case series of peripheral pigmented placoid corneal endotheliopathy (PPPCE).
Methods: A retrospective chart review of patient demographics, medical histories, and clinical characteristics was performed. Examinations included the following specialized imaging modalities: slit-lamp photography, gonioscopy, high-frequency ultrasound biomicroscopy, and anterior segment ocular coherence tomography. A PubMed and multiple corneal textbook literature search using the key words cornea, pigment, plaque, and endothelium revealed that no similar cases were reported.
Results: Five eyes in 4 asymptomatic female patients were affected. Their mean age was 53 years (range, 43–61 years), and 3 were of African American descent and 1 was of Hispanic descent. The PPPCE lesions had a vertical dimension of 0.2 to 1.7 mm and a horizontal dimension of 0.5 to 6.1 mm. All the PPPCE lesions were well demarcated, brown, and peripherally located on the inferior corneal endothelium. Clock-hour meridians extended from 4 to 7 o'clock, with the largest PPPCE lesion spanning 4.3 clock hours. Gonioscopy revealed distinct well-circumscribed brown-pigmented plaques adherent to the corneal endothelium with no extension beyond the trabecular meshwork. Ultrasound biomicroscopy and anterior segment ocular coherence tomography revealed the presence of hyperreflective lesions with no corneal stromal invasion, edema, or epitheliopathy. There were no synchronous anterior or posterior segment abnormalities. The PPPCE lesions have remained unchanged for a mean of 17 months (range, 8–34).
Conclusions: Four healthy patients were noted to have PPPCE lesions. Although their etiology remains unknown, PPPCE behavior, morphology, and inferior corneal location suggest an origin from iris stromal melanocytes or iris pigment epithelium.
*The New York Eye Cancer Center, New York, NY;
†Department of Ophthalmology, New York University School of Medicine, New York, NY;
‡Department of Ophthalmology, The New York Eye and Ear Infirmary, New York, NY; and
§Edward S Harkness Eye Institute, Columbia University Medical Center, New York, NY.
Reprints: Paul T. Finger, The New York Eye Cancer Center, 115 East 61st St, Suite 5B, New York City, NY 10065 (e-mail: firstname.lastname@example.org).
Supported by The Eye Cancer Foundation, Inc, New York, NY (http://eyecancerfoundation.net).
The authors have no conflicts of interest to disclose.
L.G. Chen contributed to the conception and design, acquisition of data, and analysis and interpretation of data, drafting of the article, and revising it critically for important intellectual content and final approval of the version to be published. P.T. Finger contributed to the conception and design, acquisition of data, and analysis and interpretation of data, revising the article critically for important intellectual content and final approval of the version to be published. E. Dhrami-Gavazi contributed to the acquisition of data, reviewing the article for intellectual content and final approval of the version to be published.
Received February 20, 2013
Accepted July 14, 2013