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Definition

A form of erythrokeratodermia variabilis et progressiva, a genodermatosis characterized by the coexistence of two independent skin lesions: transient erythema and hyperkeratosis that is usually localized but occasionally occurs in its generalized form. Clinical presentation varies significantly within a family and from one family to another. Palmoplantar keratoderma is present in around 50% of cases.

Acronym

EKVP1

Synonyms

Congenital familial erythrokeratodermia figurata in plaques
EKV
EKVP
Erythrokeratodermia progressive symmetric
Erythrokeratodermia variabilis
Erythrokeratodermia variabilis et progressiva
Erythrokeratodermia variabilis Mendes da Costa type
Erythrokeratodermia variabilis with erythema gyratum repens
Greither Disease
Keratosis palmoplantaris transgrediens et progrediens
PSEK
Transgrediens et progrediens palmoplantar keratoderma

Keywords

Palmoplantar keratoderma