{"orpha":63455,"name":"Paraneoplastic pemphigus","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare form of autoimmune bullous skin disease characterized by polyformative skin lesions, typically beginning on the oral mucus membranes, and generally associated with lymphoma or chronic lymphoid leukemia.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-63455/","orphanet_url":"https://www.orpha.net/en/disease/detail/63455","names":{"fr":{"name":"Pemphigus paranéoplasique","definition":"Forme rare de dermatite bulleuse auto-immune caractérisée par des lésions cutanées polyformatives, apparaissant d'abord sur les muqueuses buccales, et généralement associée à un lymphome ou à une leucémie lymphoïde chronique."},"de":{"name":"Pemphigus, paraneoplastischer","definition":"Eine seltene Form einer autoimmunen bullösen Hauterkrankung, die durch polyformative Hautläsionen gekennzeichnet ist, die typischerweise an den Mundschleimhäuten beginnen und im Allgemeinen mit Lymphomen oder chronischer lymphatischer Leukämie einhergehen."},"es":{"name":"Penfigoide paraneoplásico","definition":"Es una forma poco frecuente de enfermedad cutánea ampollosa autoinmune caracterizada por unas lesiones cutáneas poliformativas, que suelen aparecer en las membranas mucosas orales y, por lo general, se asocian a un linfoma o a una leucemia linfática crónica."},"it":{"name":"Pemfigo paraneoplastico","definition":"Il pemfigo paraneoplastico è una malattia cutanea bollosa autoimmune rara, caratterizzata da lesioni cutanee polimorfe, che si manifestano dapprima sulla mucosa orale, di solito associate al linfoma o alla leucemia linfatica cronica."},"nl":{"name":"Paraneoplastische pemfigus","synonyms":["Pemphigus paraneoplastica"],"definition":"Een zeldzame vorm van auto-immune bulleuze huidziekte, gekenmerkt door polyformatieve huidlaesies, typisch beginnend op orale mucosa, en doorgaans geassocieerd met lymfoom of chronische lymfatische leukemie."},"pt":{"name":"Pênfigo paraneoplásico"},"pl":{"name":"Pęcherzyca paraneoplastyczna","definition":"Rzadka postać autoimmunologicznej pęcherzowej choroby skóry, która charakteryzuje się wielokształtnymi zmianami skórnymi, zwykle wychodzącymi z błon śluzowych jamy ustnej. Generalnie związana jest z chłoniakiem lub przewlekłą białaczką limfatyczną."},"cs":{"name":"Paraneoplastický pemfigus"},"uk":{"name":"Паранеопластичний пемфігус","definition":"A rare form of autoimmune bullous skin disease characterized by polyformative skin lesions, typically beginning on the oral mucus membranes, and generally associated with lymphoma or chronic lymphoid leukemia."},"zh":{"name":"副肿瘤性天疱疮"},"tr":{"name":"Paraneoplastik pemfigus"}},"xrefs":[{"source":"ICD-10","code":"L10.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EB40.2","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"104197957"},{"source":"MONDO","code":"0018974","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1112570","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10057056","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":79669,"name":"Autoimmune bullous skin disease","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"31536300[PMID]"}],"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:0200097","label":"Oral mucosal blisters","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000155","label":"Oral ulcer","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0200041","label":"Skin erosion","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012191","label":"B-cell lymphoma","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100242","label":"Sarcoma","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100522","label":"Thymoma","frequency":"Frequent (79-30%)","criteria":null}]}}