Peripheral Pigmented Placoid Corneal Endotheliopathy

Abstract

To present a case series of peripheral pigmented placoid corneal endotheliopathy (PPPCE). 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. 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). 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.