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Erythrokeratoderma "en cocardes"

Orpha number ORPHA315
Synonym(s) Degos genodermatosis "en cocardes"
Prevalence <1 / 1 000 000
Inheritance
  • Autosomal dominant
Age of onset Childhood
ICD-10
  • Q82.8
OMIM -
UMLS -
MeSH -
MedDRA -
SNOMED CT
  • 239062001

Summary

Erythrokeratoderma 'en cocardes' is a rare genodermatosis characterised 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 (see this term). Onset usually occurs at birth or during early childhood. Only few cases have been described. Transmission is autosomal dominant.


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