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isolated congenital anonychia - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

isolated congenital anonychia

MONDO:0019211

Also known as:isolated anonychiaautosomal recessive nonsyndromic congenital nail disorder-4congenital anonychia

Overview

Isolated congenital anonychia is characterized by nail abnormalities ranging from onychodystrophy (dystrophic nails) to anonychia (absence of nails). Onychodystrophy-anonychia has been described in at least four generations of a family with male-to-male transmission, suggesting autosomal dominant transmission. Anonychia has been described in approximately less than 20 cases; it is likely to be transmitted as an autosomal recessive trait. Total anonychia congenita, in which all the fingernails and toenails are absent, may have an autosomal dominant inheritance pattern.

OnsetCONGENITAL

ResearchLimited published research (4 articles)

Prognosis

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 isolated congenital anonychia.

4 publications have been identified in PubMed for isolated congenital anonychia. Research spans Case Report / Case Series (75%) and Review / Meta-Analysis (25%).

Recent Publications

Tanti S (2026). [PMID: 41737087](https://pubmed.ncbi.nlm.nih.gov/41737087/). *Cureus*. [Case Report / Case Series]
Zhang Q (2025). [PMID: 40620857](https://pubmed.ncbi.nlm.nih.gov/40620857/). *Front Pediatr*. [Case Report / Case Series]
Iacoviello M (2025). [PMID: 40806473](https://pubmed.ncbi.nlm.nih.gov/40806473/). *Int J Mol Sci*. [Case Report / Case Series]
Cedirian S (2024). [PMID: 39386306](https://pubmed.ncbi.nlm.nih.gov/39386306/). *Skin Appendage Disord*. [Review / Meta-Analysis]

Data assembled from 3 of 12 sources · Last updated Sep 20, 2026, 4:06 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:79143

GARD

Genetic and Rare Diseases Info Center

GARD:10048

Questions for Your Care Team

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isolated congenital anonychia

Overview

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

Orphanet

GARD

Subtypes

Related Diseases

Questions for Your Care Team

Questions for Your Care Team