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Erythrokeratoderma ''en cocardes''

ORPHA:315· ICD-10 Q82.8

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.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal dominant
Age of onset
Childhood