{"orpha":505,"name":"Graham Little-Piccardi-Lassueur syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Graham Little syndrome","Piccardi-Lassueur-Little syndrome"],"definition":"A variant of lichen planopilaris characterized by the clinical triad of progressive cicatricial (scarring) alopecia of the scalp, follicular keratotic papules on glabrous skin, and variable alopecia of the axillae and groin.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-505/","orphanet_url":"https://www.orpha.net/en/disease/detail/505","names":{"fr":{"name":"Syndrome de Graham Little-Piccardi-Lassueur","synonyms":["Syndrome de Graham Little","Syndrome de Piccardi-Lassueur-Little"],"definition":"Le syndrome de Graham Little-Piccardi-Lassueur est une variante rare de lichen plan (LP) pilaire caractérisée par la triade clinique : alopécie cicatricielle progresive du cuir chevelu, kératosis folliculaire de la peau glabre et alopécie axillopubienne variable."},"de":{"name":"Graham Little-Piccardi-Lassueur-Syndrom","synonyms":["Picardi-Lassueur-Little-Syndrom"],"definition":"Das Graham Little-Piccardi-Lassueur-Syndrom ist eine Variante des Lichen planopilaris (s. dort) und gekennzeichnet durch die klinische Trias (i) progrediente vernarbende (cicatriciale) Alopezie der Kopfhaut, (ii) follikuläre keratotische Papeln auf der unbehaarten Haut, (iii) und unterschiedlich ausgeprägte Alopezie der Axillen und Leistenregion."},"es":{"name":"Síndrome de Graham Little-Piccardi-Lassueur","synonyms":["Síndrome de Graham Little","Síndrome de Piccardi-Lassueur-Little"],"definition":"Es una variante del liquen plano pilar caracterizado por una triada clínica de alopecia cicatricial progresiva del cuero cabelludo, pápulas queratósicas foliculares en la piel lampiña, y alopecia variable en axilas e ingles."},"it":{"name":"Sindrome di Graham Little-Piccardi-Lassueur","synonyms":["Sindrome di Graham Little","Sindrome di Picardi-Lassueur-Little"]},"nl":{"name":"Syndroom van Graham Little-Piccardi-Lassueur","synonyms":["Syndroom van Graham Little","Syndroom van Piccardi-Lassueur-Little"],"definition":"Een variant van lichen planopilaris, gekarakteriseerd door de klinische triade van progressieve cicatriciële (littekenvorming) alopecie van hoofdhuid, folliculaire keratotische papels op onbehaarde huid, en variabele alopecie van oksels en liesregio."},"pt":{"name":"Síndrome Graham Little-Piccardi-Lassueur","synonyms":["Síndrome Graham Little","Síndrome Picardi-Lassueur-Little"],"definition":"A síndrome Graham Little-Piccardi-Lassueur é uma variante do líquen planopilar (ver este termo), caracterizada pela tríade clínica de alopécia cicatricial progressiva do couro cabeludo, pápulas foliculares queratóticas na pele glabra, e alopécia variável das axilas e virilhas."},"pl":{"name":"Zespół Grahama Little'a,Piccardi i Lassueura","synonyms":["Zespół Grahama Little'a","Zespół Piccardi, Lassueura i Little'a"]},"cs":{"name":"Graham Littleův-Piccardiho-Lassueurův syndrom","synonyms":["Graham Littleův syndrom","Piccardiho-Lassueurův-Littleův syndrom","Graham Little-Piccardi-Lassueurův syndrom","Piccardi-Lassueur-Littleův syndrom"]},"uk":{"name":"Синдром Грема Літтла-Піккарді-Лассюра","synonyms":["Синдром Грема Літтла","Синдром Піккарді-Лассюра-Літтла"],"definition":"A variant of lichen planopilaris characterized by the clinical triad of progressive cicatricial (scarring) alopecia of the scalp, follicular keratotic papules on glabrous skin, and variable alopecia of the axillae and groin."},"zh":{"name":"Graham Little-Piccardi-Lassueur综合征","synonyms":["Piccardi-Lassueur-Little综合征"]},"tr":{"name":"Graham Little-Piccardi-Lassueur sendromu","synonyms":["Graham Little sendromu","Piccardi-Lassueur-Little sendromu"]}},"xrefs":[{"source":"ICD-10","code":"L66.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EA91.2","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"74918330"},{"source":"MONDO","code":"0018858","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4273658","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"3195","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":79364,"name":"Alopecia","classifications":[187]},{"orpha":254370,"name":"Rare cutaneous lichen planus","classifications":[187]}],"children":[],"genes":[{"symbol":"HLA-DRA","name":"major histocompatibility complex, class II, DR alpha","gene_type":"gene with protein product","association":"Major susceptibility factor in","status":"Not yet assessed","source":null,"hgnc":"4947","ensembl":"ENSG00000204287","omim":"142860","loci":["6p21.32"]}],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult"],"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:0001596","label":"Alopecia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0007468","label":"Perifollicular hyperkeratosis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002215","label":"Sparse axillary hair","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002225","label":"Sparse pubic hair","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002209","label":"Sparse scalp hair","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0100725","label":"Lichenification","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000989","label":"Pruritus","frequency":"Frequent (79-30%)","criteria":null}]}}