vitalwiki

Erythrokeratoderma variabilis progressiva

ORPHA:308166

Definition

Erythrokeratoderma variabilis progressiva (EKVP) is a type of erythrokeratoderma characterized by the association of hyperkeratosis and erythema in persistent, although sometimes variable, circumscribed lesions. Progressive symmetric erythrokeratoderma (PSEK) and erythrokeratoderma variabilis (EKV) are probably no longer two distinctive diseases but rather the two clinical manifestations of a same disease, now known as EKVP.

Prevalence
<1 / 1 000 000
Age of onset
Childhood, Infancy, Neonatal