{"orpha":308166,"name":"Erythrokeratoderma variabilis progressiva","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":[],"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.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-308166/","orphanet_url":"https://www.orpha.net/en/disease/detail/308166","names":{"fr":{"name":"Erythrokeratodermie variable progressive","definition":"Type d'érythrokératodermie caractérisé par l'association d'hyperkératose et d'érythème dans des lésions circonscrites persistantes, bien que parfois variables. L'érythrokératodermie symétrique progressive (EKSP) et l'érythrokératodermie variable (EKV) ne correspondent probablement plus à deux entités distinctes, mais à deux formes cliniques d'une même maladie, désormais connue sous le nom d'EKVP."},"de":{"name":"Erythrokeratodermie, progressive variable"},"es":{"name":"Eritroqueratodermia variable progresiva","definition":"La eritroqueratodermia variable progresiva (EQVP) es un tipo de eritroqueratodermia caracterizada por la asociación de hiperqueratosis y eritema en lesiones circunscritas persistentes, aunque en ocasiones pueden ser variables. Probablemente, la eritroqueratodermia simétrica progresiva (EQSP) y la eritroqueratodermia variable (EQV) no representan dos enfermedades diferenciadas, sino dos patrones clínicos de una misma enfermedad, conocida ahora como EQVP."},"it":{"name":"Eritrocheratoderma variabilis progressiva","definition":"L'eritrocheratodermia varibile progressiva (EKVP) è una forma di eritrocheratodermia caratterizzata dall'associazione tra ipercheratosi ed eritema, che si manifesta con lesioni circoscritte e persistenti, occasionalmente variabili. Si ritiene che l'eritrocheratodermia simmetrica progressiva (PSEK) e l'eritrocheratodermia variabile (EKV) non siano due malattie distinte, ma quadri clinici della stessa malattia, attualmente nota come EKVP."},"nl":{"name":"Erythrokeratodermie variabilis progressiva","definition":"Erythrokeratoderma variabilis progressiva (EKVP) is een type van erytrokeratodermie dat gekarakteriseerd wordt door de associatie van hyperkeratose en erytheem in aanhoudende, hoewel soms variabele, omrande letsels. Progressieve symmetrische erytrokeratodermie (PSEK) en erythrokeratoderma variabilis (EKV) zijn waarschijnlijk niet langer twee aparte ziekten meer eerder de twee klinische manifestaties van eenzelfde ziekte, nu gekend als EKVP."},"pt":{"name":"Eritoqueratodermia variabilis progressiva"},"pl":{"name":"Erytrokeratodermia zmienna postępująca"},"cs":{"name":"Erythrokeratoderma variabilis progressiva"},"uk":{"name":"Еритрокератодермія варіабельна прогресуюча","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."},"zh":{"name":"变异性红皮角化病","definition":"进行性变异性红斑角皮症（EKVP）是一种以角化过度和红斑为特征的持续性病变，也可表现为可变性局限性病灶。进行性对称性红斑角皮症（PSEK）和变异性红斑角皮症（EKV）可能不再是两种不同的疾病，而是同一疾病的两种临床表现，现在称为EKVP。"},"tr":{"name":"Eritrokeratoderma variabilis progresiva"}},"xrefs":[{"source":"MONDO","code":"0017681","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"17851","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5681068","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10923","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":79355,"name":"Erythrokeratoderma","classifications":[187]},{"orpha":183438,"name":"Genetic erythrokeratoderma","classifications":[156]}],"children":[{"orpha":317,"name":"Erythrokeratodermia variabilis","type":"Disease"},{"orpha":330029,"name":"Hypotrichosis-deafness syndrome","type":"Disease"},{"orpha":316,"name":"Progressive symmetric erythrokeratodermia","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Childhood","Infancy","Neonatal"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.046,"area":"France","status":"Validated","source":"24393603[PMID]_[EXPERT]"}],"phenotypes":null}