Benign concentric annular macular dystrophy

Definition

Benign concentric annular macular dystrophy (BCAMD) is a progressive autosomal dominant macular dystrophy characterized by parafoveal hypopigmentation followed by a retinitis pigmentosa-like phenotype (nyctalopia and peripheral vision loss) with a bullBs eye configuration.

Also known as retinitis pigmentosa 91 — per MONDO

Also identified as

• DOID 0061106 per MONDO
• MESH C537833 per MONDO
• OMIM 153870 per MONDO
• Orphanet 251287 per MONDO
• SCTID 719520001 per MONDO
• UMLS C5561925 per MONDO