Odonto-onycho-dermal dysplasia
Alerts and Notices
Synopsis

OODD typically manifests at an early age. Life expectancy ranges from normal to slightly reduced.
Codes
ICD10CM:Q82.4 – Ectodermal dysplasia (anhidrotic)
SNOMEDCT:
403762003 – Odonto-onycho-dermal dysplasia
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Witkop syndrome – An autosomal dominant disorder that presents with absent or malformed primary and/or secondary teeth, spoon-shaped nails, brittle scalp hair, and oral cleft palate.
- Congenital absence of teeth with taurodontia and sparse hair – An autosomal recessive disorder that presents with prism-shaped primary and/or secondary molars.
- Hypohidrotic ectodermal dysplasia
- Odontotrichomelic syndrome
- Schöpf-Schulz-Passarge syndrome
- Papillon-Lefèvre syndrome
Best Tests
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Management Pearls
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Therapy
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References
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Last Reviewed:08/17/2020
Last Updated:01/20/2022
Last Updated:01/20/2022