Hereditary Benign Intraepithelial Dyskeratosis

**Semantic type:** Neoplastic Process

**Definition:** A rare autosomal dominant disorder with high penetrance that affects the limbal conjunctiva or oral mucosa. 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 elevated epithelial dyskeratotic plaques in the limbal conjunctiva or oral cavity. Epithelial dysplasia is absent.

**Synonyms:** - HBID - Witkop-Von Sallmann Disease