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Erythrokeratoderma ''en cocardes''

Disease definition

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.


  • 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: -
  • GARD: 1722
  • MedDRA: -
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