syndromic disease hereditary benign intraepithelial dyskeratosis https://www.malacards.org/card/dyskeratosis_hereditary_benign_intraepithelial icd11.foundation:2059594980 A rare genetic disorder with an autosomal dominant pattern of inheritance with variable penetrance. It was initially described among Native Americans belonging to the Haliwa-Saponi tribe of northeastern North Carolina. It is caused by a duplication of chromosomal DNA at 4q35. Clinical signs present in early childhood and include asymptomatic plaques of the epibulbar conjunctivae and oral mucosa. Clinical progression of the plaques to malignancy has not been reported. OMIM:127600 MONDO:0007486 GARD:0017524 Orphanet:352657 dyskeratosis, hereditary benign intraepithelial Witkop-Von Sallmann disease SCTID:400014002 MESH:C562551 MEDGEN:75588 NCIT:C3940 HBID hereditary benign corneal intraepithelial dyskeratosis UMLS:C0265966 superficial corneal dystrophy