{"orpha":254478,"name":"Lichen planus pemphigoides","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["LP pemphigoides"],"definition":"Lichen planus (LP) pemphigoides is a rare cross-over syndrome between lichen planus and bullous pemphigoid.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-254478/","orphanet_url":"https://www.orpha.net/en/disease/detail/254478","names":{"fr":{"name":"Lichen plan pemphigoïde","synonyms":["LP pemphigoïde"],"definition":"Le lichen plan (LP) pemphigoïde est un rare syndrome à cheval entre le LP et la pemphigoïde bulleuse (PB;)."},"de":{"name":"Lichen planus pemphigoides","synonyms":["LP, pemphigoider"],"definition":"Der Lichen planus (LP) pemphigoides ist ein seltenes Syndrom mit den Zeichen des Lichen planus und des Bullösen Pemphigoids.(s. diese Termini)."},"es":{"name":"Liquen plano penfigoide","definition":"El liquen plano (LP) penfigoide es un síndrome raro cruzado entre el liquen plano y el penfigoide bulloso (consulte estos términos)."},"it":{"name":"Lichen planus pemfigoide","synonyms":["LP pemfigoide"],"definition":"Il lichen piano (LP) pemfigoide è una rara sindrome che presenta caratteristiche di sovrapposizione tra il lichen piano e il lichen pemfigoide bolloso (si veda questo termine)."},"nl":{"name":"Lichen planus pemphigoides","synonyms":["LP pemphigoides"],"definition":"Lichen planus (LP) pemphigoides is een zeldzaam cross-over-syndroom tussen lichen planus en bulleuze pemfigoïde (zie deze termen)."},"pt":{"name":"Líquen plano penfigoide","synonyms":["LP penfigoide"],"definition":"O líquen plano (LP) penfigóide é uma síndrome rara de transição entre líquen plano e penfigóide bolhoso (ver estes termos)."},"pl":{"name":"Liszaj płaski pemfingoidalny","synonyms":["LP pemfingoidalny"]},"cs":{"name":"Pemfigoidní lichen planus","synonyms":["Pemfigoidní LP"]},"uk":{"name":"Плескатий лишай пемфігоїдний","synonyms":["LP pemphigoides"],"definition":"Lichen planus (LP) pemphigoides is a rare cross-over syndrome between lichen planus and bullous pemphigoid (see these terms)."},"zh":{"name":"天疱疮样扁平苔藓","synonyms":["天疱疮样LP"],"definition":"扁平苔藓类天疱疮是一种罕见的介于扁平苔藓和大疱性类天疱疮（见该类词条）之间的交叉综合征。"},"tr":{"name":"Liken planus pemphigoides","synonyms":["LP pemfigoidleri"]}},"xrefs":[{"source":"ICD-10","code":"L43.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EB41.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1373932539"},{"source":"MONDO","code":"0016775","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0406369","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"12677","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10087934","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":[],"onset":["All ages"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":100.0,"area":"Worldwide","status":"Validated","source":"23237497[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-10-21","source":"23331194[PMID]_23626545[PMID]","terms":[{"hpo":"HP:0000962","label":"Hyperkeratosis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0007535","label":"Hypopigmented streaks","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0100725","label":"Lichenification","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0200037","label":"Skin vesicle","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0011830","label":"Abnormal oral mucosa morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000989","label":"Pruritus","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008066","label":"Abnormal blistering of the skin","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001597","label":"Abnormal nail morphology","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000498","label":"Blepharitis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000509","label":"Conjunctivitis","frequency":"Very rare (<4-1%)","criteria":null}]}}