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ORPHA:167759Category
What it is
A group of rare disorders comprises dentinogenesis imperfecta and dentin dysplasia characterized by abnormal dentin structure affecting either the primary or both the primary and secondary dentitions. Clinically, the teeth are discolored and exhibit structural defects, such as bulbous crowns and small pulp chambers on radiographs. The underlying mineralization defect often results in shearing of the overlying enamel, leaving weakened dentin exposed and prone to wear.
Key facts
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Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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