label: Conjunctival Hereditary Benign Intraepithelial Dyskeratosis
label_lc: conjunctival hereditary benign intraepithelial dyskeratosis
description: A rare autosomal dominant disorder with high penetrance that affects the limbal conjunctiva. It is almost exclusively encountered in Native Americans belonging to the Haliwa-Saponi tribe of northeastern North Carolina and is caused by a duplication in chromosome 4q35. It is characterized by the presence of frequently bilateral, elevated epithelial dyskeratotic plaques in the limbal conjunctiva. There is prominent inflammation in substantia propria. Epithelial dysplasia is absent.