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odonto-onycho-dermal dysplasia - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

odonto-onycho-dermal dysplasia

MONDO:0009773

Also known as:ectodermal dysplasiaOODDodontoonychodermal dysplasia

Find Clinical TrialsTrials

Overview

A form of ectodermal dysplasia characterized by hyperkeratosis and hyperhidrosis of the palms and soles, atrophic malar patches, hypodontia, conical teeth, onychodysplasia, and dry and sparse hair.

GeneticsAutosomal recessive · WNT10A (chr 2)

Clinical Trials7 registered · 5 recruiting

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include always present findings: Dystrophic fingernails, Widely spaced primary teeth, Short nail, and Agenesis of permanent teeth; and common findings: Sparse eyebrow, Dry skin, Smooth tongue, and Ridged nail and others. 32 total HPO annotations.

Organ System	Phenotype Count	Example Features
Skin	13	Nail dysplasia, Erythema, Dry skin

Genetic Landscape

WNT10A function has not been fully characterized.

Odonto-onycho-dermal dysplasia is associated with mutations in the WNT10A gene on chromosome 2.

Diagnosis

Genetic testing for WNT10A is available. Testing is considered confirmatory for diagnosis.

Biomarker and diagnostic research for odonto-onycho-dermal dysplasia has been reported in the published literature.

Prognosis

Phenotype severity distribution: 4 always present features, 17 common features.

Estimated prevalence: <1 in 1,000,000 (VERY_RARE).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

7 clinical trials registered, 5 recruiting. Interventions under study include other interventions, medical devices, and biologic therapy. Pipeline includes 1 PHASE2, 1 NA. Research is sponsored by a mix of industry and academic institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT04980638](https://clinicaltrials.gov/study/NCT04980638)

Data assembled from 7 of 12 sources · Last updated Sep 19, 2026, 4:33 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:257980

Orphanet

European rare disease database

Orphanet:2721

GARD

Genetic and Rare Diseases Info Center

GARD:4054

Questions for Your Care Team

→Should my partner and I meet with a genetic counselor to discuss carrier status and family planning?
→Are any of the 7 active clinical trials a good fit for my situation?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

← Back to All Diseases

Research Type	Count	% of Total
Research summaries	25	35%
Patient case studies	19	27%
Laboratory research	17	24%
Disease patterns and progression	7	10%
Testing and diagnosis research	2	3%
New treatment approaches	1	1%

Kisho

odonto-onycho-dermal dysplasia

Overview

Clinical Presentation

Genetic Landscape

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

OMIM

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications

odonto-onycho-dermal dysplasia

Overview

Clinical Presentation

Genetic Landscape

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

OMIM

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications