{"orpha":98842,"name":"Lymphomatoid papulosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["LyP"],"definition":"Lymphomatoid papulosis (LyP) is a rare cutaneous condition characterized by chronic, recurrent, and self-regressing papulonodular skin eruptions. It belongs to the spectrum of primary cutaneous CD30+ lymphoproliferative disorders, along with primary cutaneous anaplastic large cell lymphoma (primary C-ALCL) with which it shares overlapping clinical and histopathologic features.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-98842/","orphanet_url":"https://www.orpha.net/en/disease/detail/98842","names":{"fr":{"name":"Papulose lymphomatoïde","definition":"La papulose lymphomatoïde (LyP) est une maladie cutanée rare caractérisée par des éruptions chroniques, récurrentes et à rémission spontanée. Elle fait partie du spectre des maladies lymphoprolifératives cutanées CD30+, de même que les lymphomes cutanés primitifs anaplasiques à grandes cellules (C-ALCL primaire) avec lesquels elle partage des signes cliniques et histopathologiques."},"de":{"name":"Papulose, lymphomatoide","synonyms":["LyP"],"definition":"Die lymphomatoide Papulose ist eine seltene Hautkrankheit gekennzeichnet durch chronische, rekurrente und von selbst wieder abflauende papulonoduläre Hauteffloreszenzen. Sie gehört zum Spektrum der primären kutanen CD30-positiven lymphoproliferatien Erkrankungen zusammen mit dem primären kutanen anaplastischen Großzell-Lymphom (primäres C-ALCL; s. diesen Terminus), mit dem sie überlappende klinische und histopathologische Merkmale teilt."},"es":{"name":"Papulosis linfomatoide","synonyms":["LyP"],"definition":"La papulosis linfomatoide (PL) es una enfermedad cutánea rara caracterizada por erupciones cutáneas pápulo-nodulares crónicas, recurrentes y autoinvolutivas. Pertenece al espectro de los procesos linfoproliferativos cutáneos primarios CD30+, junto con el linfoma anaplásico de células grandes, tipo cutáneo primario (LACG-C primario; véase este término), con el que comparte rasgos clínicos e histopatológicos."},"it":{"name":"Papulosi linfomatoide","synonyms":["LyP"],"definition":"La papulosi linfomatoide (LyP) è una malattia cutanea rara caratterizzata da eruzioni cutanee papulo-nodulari croniche ricorrenti che regrediscono spontaneamente. Appartiene allo spettro delle malattie linfoproliferative CD30+ cutanee primitive, insieme al linfoma anaplastico cutaneo primitivo a cellule grandi (C-ALCL primitivo; si veda questo termine), con cui condivide i segni clinici ed istopatologici."},"nl":{"name":"Lymfomatoïde papulose","synonyms":["LyP"],"definition":"Lymfomatoïde papulose (LyP) is een zeldzame huidaandoening die wordt gekarakteriseerd door chronische, terugkerende, en zelf afnemende papulo-nodulaire huiduitslag. Het behoort tot het spectrum van primaire CD30+ lymfoproliferatieve huidaandoeningen, samen met primair cutaan anaplastisch grootcellig lymfoom (primair C-ALCL; zie deze term), waarmee het klinische en histopathologische overeenkomsten vertoont."},"pt":{"name":"Papulose linfomatóide"},"pl":{"name":"Grudkowatość limfoidalna","synonyms":["LyP"]},"cs":{"name":"Lymfomatoidní papulóza","synonyms":["LyP"]},"uk":{"name":"Лімфоматоїдний папулез","synonyms":["LyP"],"definition":"Lymphomatoid papulosis (LyP) is a rare cutaneous condition characterized by chronic, recurrent, and self-regressing papulonodular skin eruptions. It belongs to the spectrum of primary cutaneous CD30+ lymphoproliferative disorders, along with primary cutaneous anaplastic large cell lymphoma (primary C-ALCL; see this term) with which it shares overlapping clinical and histopathologic features."},"zh":{"name":"淋巴瘤样丘疹病","synonyms":["LyP"],"definition":"淋巴瘤样丘疹病(LYP)是一种罕见的皮肤疾病，其特征是慢性、复发性和自限性的结节性皮疹。它属于原发性皮肤CD30+淋巴增生性疾病，与原发性皮肤间变性大细胞淋巴瘤(原发性C-ALCL；见该词条)具有重叠的临床和组织病理学特征。"},"tr":{"name":"Lenfomatoid papüloz","synonyms":["LyP"]}},"xrefs":[{"source":"ICD-10","code":"C86.6","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"2B03.1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1791207220"},{"source":"MONDO","code":"0020326","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D017731","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0206182","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"6944","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10056670","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":541,"name":"Primary cutaneous CD30+ T-cell lymphoproliferative disease","classifications":[187,194,202,233]}],"children":[],"genes":[{"symbol":"NPM1","name":"nucleophosmin 1","gene_type":"gene with protein product","association":"Part of a fusion gene in","status":"Assessed","source":"25349176[PMID]","hgnc":"7910","ensembl":"ENSG00000181163","omim":"164040","loci":["5q35.1"]},{"symbol":"TYK2","name":"tyrosine kinase 2","gene_type":"gene with protein product","association":"Part of a fusion gene in","status":"Assessed","source":"25349176[PMID]","hgnc":"12440","ensembl":"ENSG00000105397","omim":"176941","loci":["19p13.2"]}],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}