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

Rare Disease

odonto-onycho dysplasia-alopecia syndrome

MONDO:0017134

Also known as:odonto onycho dysplasia with alopecia

Overview

Odonto-onycho dysplasia-alopecia syndrome is a rare, genetic ectodermal dysplasia syndrome characterized by almost total alopecia with only sparse, thin, brittle, slow-growing scalp hair, fair and sparse eyebrows and eyelashes, absent axillary and pubic hair, fragile and brittle fingernails, thick and brittle toenails (both with a subungual corneal layer), hypodontia, microdontia, widely spaced teeth with hypoplastic enamel, mild palmoplantar keratosis, cafe-au-lait spots and areolae anomalies. There have been no further descriptions in the literature since 1985.

ResearchLimited published research (2 articles)

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 very common findings: Microdontia, Tooth malposition, Palmoplantar keratoderma, and Abnormal fingernail morphology and others.

Organ System	Phenotype Count	Example Features
Skin	2	Palmoplantar keratoderma, Alopecia
Arms and legs

Prognosis

Phenotype severity distribution: 11 very 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.

No clinical trials have been registered for odonto-onycho dysplasia-alopecia syndrome.

2 publications have been identified in PubMed for odonto-onycho dysplasia-alopecia syndrome. Research spans Review / Meta-Analysis (100%).

Recent Publications

Morandini AC (2025). [PMID: 40083426](https://pubmed.ncbi.nlm.nih.gov/40083426/). *Front Pediatr*. [Review / Meta-Analysis]
Van Steensel MAM (2025). [PMID: 40689430](https://pubmed.ncbi.nlm.nih.gov/40689430/). *Ann Hum Genet*. [Review / Meta-Analysis]

Data assembled from 4 of 12 sources · Last updated Sep 20, 2026, 5:40 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:2722

GARD

Genetic and Rare Diseases Info Center

GARD:4051

Questions for Your Care Team

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odonto-onycho dysplasia-alopecia syndrome

Overview

Clinical Presentation

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

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