Preferred Label : Erythrokeratoderma variabilis et progressiva;
ICD-11 definition : Mendes da Costa type erythrokeratodermia variabilis (EKV) is a genodermatosis characterised
by the appearance of two independent skin lesions: transient figurate erythematous
patches and hyperkeratosis that is usually localised but occasionally occurs in its
generalised form, generally during the first year of life.;
ICD-11 inclusion : Mendes da Costa syndrome; Erythrokeratoderma progressiva symmetrica; Erythrokeratoderma variabilis;
Origin ID : 886266813;
Currated CISMeF NLP mapping
Mendes da Costa type erythrokeratodermia variabilis (EKV) is a genodermatosis characterised
by the appearance of two independent skin lesions: transient figurate erythematous
patches and hyperkeratosis that is usually localised but occasionally occurs in its
generalised form, generally during the first year of life.
