{"orpha":141103,"name":"Nasal dermoid cyst","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":["Nasal dermoid sinus cyst"],"definition":"A rare otorhinolaryngological malformation characterized by a dermoid cyst along the nasal dorsum or glabella, lined by keratinized squamous epithelium and containing intraluminal keratin and mature adnexal structures, such as hair follicles, sebaceous and sweat glands. The majority of nasal dermoid cysts are superficial, rarely they extend intracranially. The cysts are typically benign but are susceptible to recurrent infections that may progress to osteomyelitis, meningitis or an intracranial abscess.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-141103/","orphanet_url":"https://www.orpha.net/en/disease/detail/141103","names":{"fr":{"name":"Kyste dermoïde nasal","definition":"Malformation otorhinolaryngologique rare caractérisée par un kyste dermoïde le long du dorsum nasal ou de la glabelle, tapissé d'un épithélium kératinisé d'aspect pavimenteux, constitué de kératine intraluminale et des masses annexielles matures, telles que des follicules pileux, des glandes sébacées et sudoripares. La majorité des kystes dermoïdes nasaux sont superficiels et s'étendent rarement à la zone intracrânienne. Généralement bénins, ils peuvent être le siège d'infections récurrentes pouvant évoluer vers une ostéomyélite, une méningite ou un abcès intracrânien."},"de":{"name":"Dermoidzyste, nasale","definition":"Eine seltene otorhinolaryngologische Fehlbildung, die durch eine Dermoidzyste entlang des Nasenrückens oder der Glabella gekennzeichnet ist, die von keratinisiertem Plattenepithel ausgekleidet ist und intraluminales Keratin und reife adnexale Strukturen wie Haarfollikel, Talg- und Schweißdrüsen enthält. Die Mehrzahl der nasalen Dermoidzysten ist oberflächlich, selten dehnen sie sich intrakraniell aus. Die Zysten sind typischerweise gutartig, aber die Patienten neigen zu rezidivierenden Infektionen, die sich zu Osteomyelitis, Meningitis oder einem intrakraniellen Abszess entwickeln können."},"es":{"name":"Quiste dermoide nasal","synonyms":["Quiste dermoide de la cavidad nasal","Quiste dermoide nasal del sino"],"definition":"Es una malformación otorrinolaringológica poco frecuente caracterizada por un quiste dermoide a lo largo del dorso nasal o la glabela, recubierto por epitelio escamoso queratinizado y que contiene queratina intraluminal y estructuras anexas maduras, tales como folículos pilosos, glándulas sebáceas y sudoríparas. La mayoría de los quistes dermoides nasales son superficiales, aunque excepcionalmente se extienden intracranealmente. Los quistes son típicamente benignos, aunque son susceptibles a infecciones recurrentes que pueden progresar a osteomielitis, meningitis o a un absceso intracraneal."},"it":{"name":"Cisti dermoide nasale","definition":"Si tratta di una malformazione rara di interesse otorinolaringoiatrico, caratterizzata da una cisti dermoide localizzata sul dorso del naso o sulla glabella, rivestita da un epitelio squamoso cheratinizzato, contenente cheratina intraluminale e annessi maturi (follicoli piliferi, ghiandole sebacee e sudoripare). La maggior parte delle cisti dermoidi nasali è superficiale; solo raramente si osserva un interessamento intracranico. Di solito le cisti sono benigne, ma sono suscettibili alle infezioni ricorrenti che possono progredire nell'osteomielite, nella meningite o negli ascessi intracranici."},"nl":{"name":"Nasale dermoïdcyste","synonyms":["Nasale dermoïd-sinuscyste"],"definition":"Een zeldzame otorinolaryngologische malformatie gekarakteriseerd door een dermoïdcyste langs de neusrug of de glabella, bekleed met gekeratiniseerd squameus epitheel en binnenin intraluminaal keratine en volgroeide adnexale structuren, zoals haarfollikels, sebaceuze klieren en zweetklieren. De meerderheid van de nasale dermoïdcysten is oppervlakkig, en maar zelden is er intracraniële uitbreiding. De cysten zijn doorgaans goedaardig maar vatbaar voor recurrente infecties die kunnen evolueren tot osteomyelitis, meningitis of een intracranieel abces."},"pt":{"name":"Quisto dermóide nasal"},"pl":{"name":"Torbiel skórzasta nosa","synonyms":["Torbiel skórzasta zatoki nosowej"]},"cs":{"name":"Nazální dermoidní cysta","synonyms":["Nazální dermoidní cysta dutin"]},"uk":{"name":"Дермоїдна кіста носа","synonyms":["Кіста дермоїдного синуса носа"],"definition":"A rare otorhinolaryngological malformation characterized by a dermoid cyst along the nasal dorsum or glabella, lined by keratinized squamous epithelium and containing intraluminal keratin and mature adnexal structures, such as hair follicles, sebaceous and sweat glands. The majority of nasal dermoid cysts are superficial, rarely they extend intracranially. The cysts are typically benign but are susceptible to recurrent infections that may progress to osteomyelitis, meningitis or an intracranial abscess."},"zh":{"name":"鼻部皮样囊肿","synonyms":["鼻皮样窦囊肿"],"definition":"一种罕见的耳鼻喉畸形，其特征是沿鼻背或眉间出现皮样囊肿，覆以角化鳞状上皮，内含管腔内角蛋白和成熟附件结构，如毛囊、皮脂腺和汗腺。大多数鼻皮样囊肿为浅表性，少有颅内延伸。囊肿常为良性，但易反复感染，可能发展为骨髓炎、脑膜炎或颅内脓肿。"},"tr":{"name":"Nazal dermoid kist","synonyms":["Nazal dermoid sinüs kisti"]}},"xrefs":[{"source":"OMIM","code":"600679","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"Q18.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"DA05.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true,"icd11_uri":"null"},{"source":"MONDO","code":"0010921","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0339852","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98036,"name":"Rare otorhinolaryngologic disease"},"parents":[{"orpha":155835,"name":"Cysts and fistulae of the face and oral cavity","classifications":[147,200]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}