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erythrokeratoderma en cocardes - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

erythrokeratoderma en cocardes

MONDO:0017836

Also known as:Degos genodermatosisDegos genodermatosis "en cocardes"Degos 'en cocarde' erythrokeratoderma

Overview

Erythrokeratoderma 'en cocardes' is a rare genodermatosis characterized by circumscribed target-like (or 'en cocardes') erythematous hyperkeratotic lesions. These lesions, which remit and recur, affect the trunk and extremities and are accompanied by scaly plaques evocative of erythrokeratoderma variabilis. Onset usually occurs at birth or during early childhood. Only few cases have been described. Transmission is autosomal dominant.

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: Thickened, rough skin (hyperkeratosis), Skin color changes (abnormality of skin pigmentation), Neoplasm of the skin, and Papule; and sometimes findings: Neoplasm.

Organ System	Phenotype Count	Example Features
Skin	4	Thickened, rough skin (hyperkeratosis), Skin color changes (abnormality of skin pigmentation), Neoplasm of the skin

Prognosis

Phenotype severity distribution: 4 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 erythrokeratoderma en cocardes.

10 publications have been identified in PubMed for erythrokeratoderma en cocardes. Research spans Case Report / Case Series (70%) and Review / Meta-Analysis (30%).

Research Type	Count	% of Total
Patient case studies	7	70%

Data assembled from 4 of 12 sources · Last updated Sep 17, 2026, 9:26 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:315

GARD

Genetic and Rare Diseases Info Center

GARD:1722

Questions for Your Care Team

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erythrokeratoderma en cocardes

Overview

Clinical Presentation

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications

erythrokeratoderma en cocardes

Overview

Clinical Presentation

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications