{"orpha":33408,"name":"Bullous lichen planus","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Bullous lichen planus is a variant of rare lichen planus characterized by the development of vesico-bullous lesions.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-33408/","orphanet_url":"https://www.orpha.net/en/disease/detail/33408","names":{"fr":{"name":"Lichen bulleux","definition":"Le lichen plan bulleux est une forme rare du lichen plan caractérisé par des lésions vésiculobulleuses."},"de":{"name":"Lichen bullosus","synonyms":["Lichen planus bullosus"],"definition":"Der Bullöse Lichen planus, eine seltene Variante des Lichen planus (s. dort), ist gekennzeichnet durch das Auftreten blasig-bullöser Läsionen."},"es":{"name":"Liquen plano ampollar","definition":"El liquen plano ampollar es una variante del liquen plano raro (consulte este término) que se caracteriza por el desarrollo de lesiones vesículo-ampollares."},"it":{"name":"Lichen planus bolloso","definition":"Il lichen piano bolloso è una variante del lichen piano raro (si veda questo termine), caratterizzata dallo sviluppo di lesioni vescicolo-bollose."},"nl":{"name":"Bulleuze lichen planus","definition":"Bulleuze lichen planus is een variant van zeldzame lichen planus (zie deze term), gekenmerkt door de ontwikkeling van vesico-bulleuze laesies."},"pt":{"name":"Líquen plano bolhoso","definition":"O líquen plano bolhoso é uma variante do raro líquen plano (ver este termo), caracterizado pelo desenvolvimento de lesões vesico-bolhosas."},"pl":{"name":"Liszaj płaski pęcherzowy"},"cs":{"name":"Bulózní lichen planus"},"uk":{"name":"Бульозний плоский лишай","definition":"Bullous lichen planus is a variant of rare lichen planus (see this term) characterized by the development of vesico-bullous lesions."},"zh":{"name":"大疱性扁平苔癣"},"tr":{"name":"Büllöz liken planus"}},"xrefs":[{"source":"ICD-10","code":"L43.1","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"EA91.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"606709958"},{"source":"MONDO","code":"0018056","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0023648","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10056960","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":254370,"name":"Rare cutaneous lichen planus","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Autosomal dominant","Not applicable"],"onset":["Childhood"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0008066","label":"Abnormal blistering of the skin","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0100783","label":"Breast aplasia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0100725","label":"Lichenification","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0200034","label":"Papule","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000989","label":"Pruritus","frequency":"Occasional (29-5%)","criteria":null}]}}