{"orpha":492,"name":"Proliferating trichilemmal cyst","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare large, multinodular, usually benign, tumor that is generally located in the posterior part of the scalp in aged women (over 50 years). It first appears as a painless nodule that later grows into a solid or partially cystic tumor that is mobile over the underlying subcutaneous tissues. It can present ulceration, inflammation or even bleeding and can cause necrosis of the adjacent tissues.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-492/","orphanet_url":"https://www.orpha.net/en/disease/detail/492","names":{"fr":{"name":"Kyste trichilemmal proliférant","definition":"Le kyste trichilemmal proliférant est une tumeur large, multinodulaire, rare et généralement bénigne, habituellement située dans la partie postérieure du cuir chevelu chez les femmes âgées (> 50 ans). Il se manifeste tout d'abord comme un nodule indolore progressant ultérieurement en une tumeur solide ou partiellement kystique, mobile sur les tissus sous-cutanés sous-jacents. Il peut présenter une ulcération, une inflammation ou même un saignement, et peut causer une nécrose des tissus adjacents."},"de":{"name":"Trichilemmalzyste, proliferierende","definition":"Der Proliferierende trichilemmale Tumor ist ein seltener, großer, multinodulärer, in der Regel benigner Tumor, der meist im hinteren Bereich der Kopfhaut liegt und bei älteren Frauen (über 50 Jahre) auftritt. Er erscheint anfangs als schmerzloses Knötchen, das später zu einem soliden oder teilweise zystischen, über dem subkutanen Gewebe beweglichen Tumor wächst. Der Tumor kann ulzerieren, sich entzünden oder sogar bluten und in den benachbarten Geweben Nekrosen verursachen."},"es":{"name":"Quiste triquilemal proliferante","definition":"Es un tumor grande, multinodular, normalmente benigno, que está generalmente localizado en la parte posterior del cuero cabelludo en mujeres mayores (de más de 50 años). Aparece primero como un nódulo indoloro que se convierte más tarde en un tumor sólido o parcialmente quístico, móvil bajo los tejidos subcutáneos subyacentes. Puede presentar ulceraciones, inflamación o incluso sangrado y puede causar necrosis de los tejidos adyacentes."},"it":{"name":"Cisti trichilemmale proliferativa","definition":"Il tumore trichilemmale proliferativo è un tumore esteso, multinodulare, raro, di solito benigno, localizzato generalmente nell'area posteriore del cuoio capelluto nelle donne attempate (oltre i 50 anni). Si presenza, primariamente, come un nodulo indolore che evolve successivamente in un tumore solido o parzialmente cistico, che è mobile all'interno del tessuto sottocutaneo sottostanti. Può presentare ulcerazioni, infiammazione o anche sanguinamento e può causare la necrosi dei tessuti adiacenti."},"nl":{"name":"Prolifererende trichilemmale cyste","definition":"Een zeldzame, grote, multinodulaire, meestal goedaardige tumor die doorgaans is gesitueerd op het posterieure gedeelte van de hoofdhuid bij oudere vrouwen (ouder dan 50 jaar). Het verschijnt eerst als een pijnloze knobbel die later uitgroeit tot een solide of deels cystische tumor die mobiel is over de onderliggende onderhuidse weefsels. Het kan ulceratie, inflammatie of zelfs bloeding vertonen, en kan necrose van aangrenzende weefsels veroorzaken."},"pt":{"name":"Quisto triquilémico proliferativo"},"pl":{"name":"Torbiel włosowa proliferująca"},"cs":{"name":"Proliferující trichilemální cysta"},"uk":{"name":"Проліферуюча трихілемальна кіста","definition":"A rare large, multinodular, usually benign, tumor that is generally located in the posterior part of the scalp in aged women (over 50 years). It first appears as a painless nodule that later grows into a solid or partially cystic tumor that is mobile over the underlying subcutaneous tissues. It can present ulceration, inflammation or even bleeding and can cause necrosis of the adjacent tissues."},"zh":{"name":"增生性毛根鞘囊肿"},"tr":{"name":"Çoğalan trikilemmal kist"}},"xrefs":[{"source":"ICD-10","code":"L72.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EK70.1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"150930710"},{"source":"ICD-11","code":"XH7WJ7","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"521331707"},{"source":"MONDO","code":"0018850","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2959585","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"4509","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":79386,"name":"Rare skin tumor or hamartoma","classifications":[187,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult"],"prevalence":[],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0200040","label":"Epidermoid cyst","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0200042","label":"Skin ulcer","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002209","label":"Sparse scalp hair","frequency":"Frequent (79-30%)","criteria":null}]}}