{"orpha":90396,"name":"Acral persistent papular mucinosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare chronic form of localized lichen myxedematosus characterized by the development of multiple symmetrical skin-colored mucinous papules exclusively on the extensor surface of the hands and distal forearms.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-90396/","orphanet_url":"https://www.orpha.net/en/disease/detail/90396","names":{"fr":{"name":"Mucinose papuleuse acrale persistante","definition":"La mucinose papuleuse acrale persistante est une forme chronique rare du lichen myxoedémateux localisé caractérisée par le développement de multiples papules mucineuses symétriques couleur chair exclusivement sur la face d'extension des mains et sur la partie distale des avant-bras."},"de":{"name":"Muzinose, papulöse, akral-persistierende","definition":"Die Akrale persistierende papuläre Muzinose ist eine seltene chronische Form des Lokalisierten Lichen myxoedematosus (s. dort) und gekennzeichnet durch die Entwicklung symmetrischer hautfarbener muzinöser Papeln ausschließlich an den Streckseiten der Hände und der distalen Unterarme."},"es":{"name":"Mucinosis papular acra persistente","definition":"Es una forma crónica poco frecuente de liquen mixedematoso localizado, que se caracteriza por el desarrollo de múltiples pápulas mucinosas color piel exclusivamente en las superficies extensoras de las manos y de los antebrazos distales."},"it":{"name":"Mucinosi papulosa acrale persistente","definition":"La mucinosi papulosa persistente acrale è una forma rara del lichen mixedematoso localizzato (si veda questo termine), caratterizzata dallo sviluppo di papule mucinose simmetriche multiple color carne esclusivamente sulla superficie estensoria delle mani e sulle estremità distali degli avambracci."},"nl":{"name":"Acrale persisterende papulaire mucinose","definition":"Een zeldzame, chronische vorm van gelokaliseerde lichen myxoedematosus, gekarakteriseerd door de ontwikkeling van multipele symmetrische, huidkleurige, mucineuze papels, exclusief op de strekzijde van handen en distale onderarmen."},"pt":{"name":"Mucinose papular acral persistente"},"pl":{"name":"Obwodowa przewlekła mucynoza grudkowa"},"cs":{"name":"Akrální perzistentní papulární mucinóza"},"uk":{"name":"Акральний персистуючий папульозний муциноз","definition":"A rare chronic form of localized lichen myxedematosus characterized by the development of multiple symmetrical skin-colored mucinous papules exclusively on the extensor surface of the hands and distal forearms."},"zh":{"name":"肢端持续性丘疹性黏蛋白病"},"tr":{"name":"Akral kalıcı papüler müsinoz"}},"xrefs":[{"source":"ICD-10","code":"L98.5","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EB90.1Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"712202928"},{"source":"MONDO","code":"0019581","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0406660","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":86795,"name":"Localized lichen myxedematosus","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":40.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}