ICD-11 classes
13 Diseases of the digestive system
Diseases or disorders of orofacial complex
DA07 — Disorders of tooth development or eruption
LA30.6 — Amelogenesis imperfecta

ICD-11 LA30.6 — Amelogenesis imperfecta

Amelogenesis imperfecta presents with a rare abnormal formation of the enamel or external layer of the crown of teeth. Amelogenesis imperfecta is due to the malfunction of the proteins in the enamel: ameloblastin, enamelin, tuftelin and amelogenin. People afflicted with amelogenesis imperfecta have teeth with abnormal colour: yellow, brown or grey; this disorder can afflict any number of teeth of both dentitions. The teeth have a higher risk for dental cavities and are hypersensitive to temperature changes as well as rapid attrition, excessive calculus deposition, and gingival hyperplasia.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

• Structural developmental anomalies of teeth and periodontal tissues #22405
• Syndromes with dental anomalies as a major feature #23217