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Congenital ichthyosiform erythroderma

ORPHA:79394· ICD-10 Q80.2

Definition

A rare autosomal recessive congenital ichthyosis (ARCI) characterized by generalised scaling accompanied by a more or less severe erythroderma, without blister formation.

Prevalence
1-9 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
Infancy, Neonatal