Dentin dysplasia type I

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Dentin dysplasia type I

ORPHA:99789Clinical subtype

Also called DD-I · DTDP1 · Radicular dentin dysplasia

What it is

Dentin dysplasia type I (DD-I) is a rare form of dentin dysplasia (DD) characterized by sharp conical short roots or rootless teeth.

Key facts

Prevalence
1-9 / 100 000 (Europe)
Age of onset
Childhood
Inheritance
Autosomal dominant, Autosomal recessive
Classified as
Clinical subtype

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Genes

SSUH2Disease-causing germline mutation(s)
VPS4BDisease-causing germline mutation(s) (loss of function)

ICD-10 codes

K00.5filed under a broader ICD-10 category — shared with 13 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

GARD 1807MESH C538215MONDO 0007436OMIM 621440OMIM 621559UMLS C0399379

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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