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Erythrokeratoderma ''en cocardes''

Disease definition

A rare, genetic, epidermal disorder characterized by intermittent (remitting and recurring), annular, polycyclic, target-like (or 'en cocardes') plaques with concentric rings of scaling erythema occurring on the extremities, flexural areas, and trunk. Concurrent erythrokeratoderma variabilis-like scaly plaques are commonly found in other parts of the body.

ORPHA:315

Classification level: Disorder
  • 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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