Erythrokeratoderma en cocardes

The erythrokeratodermas are a distinct but clinically variable group of rar e genodermatoses, characterized by circumscribed erythematous and hyperkera totic lesions. All attempts to establish a valid classification have been b ased on purely clinical and morphologic criteria. Erythrokeratoderma en coc ardes, also known as genodermatose en cocardes or Degos' syndrome, was firs t described by Degos in 1947. The condition is characterized by large round plaques with concentric erythema and scaling having a target configuration , which remit and recur, in addition to scaly plaques as seen in erythroker atoderma variabilis. A case of this rare genodermatosis is described.