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punctate palmoplantar keratoderma type III (AKE) - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

punctate palmoplantar keratoderma type III

MONDO:0007047

Also known as:AKEPPKP3acrokeratoelastoidosis of Costa

Overview

Acrokeratoelastoidosis of Costa is a rare dermatosis characterized by small, firm papules or plaques (resembling warts) on the sides of the hands and feet. These stationary and asymptomatic lesions appear generally at puberty, or sometimes later

GeneticsAutosomal dominant

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: Palmoplantar hyperkeratosis, Epidermal acanthosis, Fragmented elastic fibers in the dermis, and Hyperkeratotic papule; and common findings: Granulomatosis, Hypergranulosis, Yellow papule, and Orthokeratosis. 14 total HPO annotations.

Organ System	Phenotype Count	Example Features
Skin	7	Thickened, rough skin (hyperkeratosis), Palmoplantar hyperkeratosis, Hyperkeratotic papule

Prognosis

Phenotype severity distribution: 4 very common features, 4 common features.

Estimated prevalence: Unknown (Unknown prevalence).

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 punctate palmoplantar keratoderma type III.

2 publications have been identified in PubMed for punctate palmoplantar keratoderma type III. Research spans Case Report / Case Series (100%).

Recent Publications

Li Y (2026). [PMID: 41716399](https://pubmed.ncbi.nlm.nih.gov/41716399/). *Front Immunol*. [Case Report / Case Series]
Edwards TM (2024). [PMID: 39654849](https://pubmed.ncbi.nlm.nih.gov/39654849/). *JAAD Case Rep*. [Case Report / Case Series]

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 11:54 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:101850

Orphanet

European rare disease database

Orphanet:38

GARD

Genetic and Rare Diseases Info Center

GARD:125

Questions for Your Care Team

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punctate palmoplantar keratoderma type III

Overview

Clinical Presentation

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

OMIM

Orphanet

GARD

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Questions for Your Care Team

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