{"orpha":263425,"name":"Nevus of Ota","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Nevus fusculoceruleus ophthalmomaxillaris"],"definition":"Nevus of Ota is an oculodermal melanocytosis more commonly found in Asian and African populations, usually present at birth and characterized by a usually unilateral, bluish gray, patchy, speckled pigmentation (that may progressively enlarge and darken) affecting the skin of the face along the distribution of the ophthalmic and maxillary divisions of the trigeminal nerve (periorbital region, temple, forehead, malar area, nose). In 2/3 cases the ipsilateral sclera is affected. Nevus of Ota usually remains stable once adulthood is reached but an increased risk of glaucoma and uveal melanoma may be observed. Extracutaneous lesions may also occur in cornea, retina, tympanum, nasal mucosa, pharynx, palate. Nevus of Ota occurs as solitary conditions but seldom may occur together with the nevus of Ito or nevus spilus.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-263425/","orphanet_url":"https://www.orpha.net/en/disease/detail/263425","names":{"fr":{"name":"Naevus d'Ota","definition":"Mélanocytose oculodermique plus fréquente chez les populations asiatiques et africaines. Généralement congénitale, elle se manifeste par une pigmentation tachetée et mouchetée, bleuâtre-grise, généralement unilatérale (qui peut progressivement s'étendre et s'assombrir) affectant la peau du visage le long de la distribution des divisions ophtalmique et maxillaire du nerf trijumeau (région périorbitaire, tempe, front, zone malaire, nez). Dans 2/3 des cas, la sclère ipsilatérale est touchée. Le naevus d'Ota reste généralement stable à l'âge adulte, mais un risque accru de glaucome et de mélanome uvéal peut être observé. Des lésions extracutanées peuvent également apparaître au niveau de la cornée, de la rétine, du tympan, de la muqueuse nasale, du pharynx et du palais. Le naevus d'Ota se présente sous forme de lésion isolée, mais peut dans de rares cas être associé au naevus d'Ito ou au naevus spilus."},"de":{"name":"Naevus Ota","definition":"Der Naevus von Ota ist eine okulodermale Melanozytose, die häufiger in asiatischen und afrikanischen Populationen vorkommt. Er ist normalerweise angeboren und manifestiert sich durch eine meist einseitige, bläulich-graue, fleckige, gefleckte Pigmentierung (die sich nach und nach vergrößern und verdunkeln kann), der Gesichtshaut entlang der Verteilung der ophthalmischen und maxillären Versorgungsbereiche des Trigeminusnervs (Periorbitalregion, Schläfe, Stirn, Malarbereich, Nase). In 2/3 Fällen ist die ipsilaterale Sklera betroffen. Der Nävus von Ota bleibt in der Regel stabil, wenn das Erwachsenenalter erreicht ist, aber ein erhöhtes Risiko für Glaukom und Uvealmelanom kann beobachtet werden. Extrakutane Läsionen können auch in Hornhaut, Netzhaut, Tympanon, Nasenschleimhaut, Rachen, Gaumen auftreten. Normalerweise ist der Nävus von Ota ein eigenständiges Erscheinungsbild, er kann in seltenen Fällen jedoch zusammen mit dem Nävus von Ito oder Nävus spilus auftreten."},"es":{"name":"Nevo de Ota","synonyms":["Nevo fusco-cerúleo oftálmico maxilar"],"definition":"Es una melanocitosis oculo-dérmica observada con mayor frecuencia en poblaciones asiáticas y africanas. Suele estar presente al nacimiento y se caracteriza por una pigmentación gris azulada moteada y parcheada generalmente unilateral (que puede aumentar de tamaño y oscurecerse progresivamente) localizada en la cara siguiendo la distribución de las divisiones oftálmica y maxilar del nervio trigémino (región periorbital, sien, frente, área malar, nariz). En 2/3 de los casos afecta la esclerótica ipsilateral. Suele permanecer estable una vez que se alcanza la edad adulta, aunque puede asociarse con un mayor riesgo para desarrollar glaucoma y melanoma uveal. Se han descrito asimismo lesiones extracutáneas en la córnea, retina, tímpano, mucosa nasal, faringe y paladar. Suele ser una lesión solitaria, aunque en algún caso se ha descrito su coexistencia con un nevo de Ito o con un nevo spilus."},"it":{"name":"Nevo di Ota","synonyms":["Nevus fusculocoeruleus ophthalmomaxillaris"],"definition":"Il nevo di Ota è una melanocitosi oculodermica, più comune nelle popolazioni asiatiche e africane; di solito esordisce alla nascita ed è caratterizzato da pigmentazione generalmente bilaterale, di colore grigio-bluastro, a chiazze (che si allargano e si scuriscono progressivamente) che si localizzano sulla cute del viso, lungo la distribuzione della ramificazione oftalmica e mascellare del nervo trigemino (regione periorbitale, tempie, fronte, zigomi, naso). Nei due terzi dei casi è interessata monolateralmente la sclera. Di solito, il nevo di Ota si stabilizza una volta raggiunta l'età adulta, ma si associa ad un maggiore rischio di glaucoma e melanoma uveale. Possono essere presenti lesioni extracutanee, a livello della cornea, della retina, del timpano, della muscosa nasale, della faringe e del palato. Il nevo di Ota è una malattia isolata, ma raramente può associarsi a nevo di Ito o al nevo spilus."},"nl":{"name":"Naevus van Ota","synonyms":["Naevus fusculocoeruleus ophthalmomaxillaris"],"definition":"Naevus van Ota is een oculodermale melanocytose die vaker wordt aangetroffen in Aziatische en Afrikaanse populaties, die meestal aanwezig is bij de geboorte, en die gekarakteriseerd wordt door een meestal unilaterale, blauwgrijze, onregelmatige, gevlekte pigmentatie (die progressief kan groter en donkerder worden) die de huid van het gelaat treft langsheen de oftalmische en maxillaire takken van de drielingzenuw (nervus trigeminus) (periorbitale regio, slapen, voorhoofd, wangen, neus). In 2/3 van de gevallen is de ipsilaterale sclera getroffen. Naevus van Ota blijft meestal stabiel eens de volwassenheid is bereikt, maar er kan een verhoogd risico op glaucoom en uveaal melanoom waargenomen worden. Extracutane letsels kunnen ook voorkomen in het hoornvlies, het netvlies, het trommelvlies, het neusslijmvlies, de farynx en het verhemelte. Naevus van Ota komt voor als alleenstaande conditie maar uitzonderlijk kan het samen voorkomen met naevus van Ito of naevus spilus."},"pt":{"name":"Nevus de Ota"},"pl":{"name":"Znamię Oty","synonyms":["Znamię okulodermalne"]},"cs":{"name":"Otův névus","synonyms":["Nevus fusculoceruleus ophthalmomaxillaris"]},"uk":{"name":"Невус Ота","synonyms":["Фускулоцеребральний невус офтальмомаксилярний"],"definition":"Nevus of Ota is an oculodermal melanocytosis more commonly found in Asian and African populations, usually present at birth and characterized by a usually unilateral, bluish gray, patchy, speckled pigmentation (that may progressively enlarge and darken) affecting the skin of the face along the distribution of the ophthalmic and maxillary divisions of the trigeminal nerve (periorbital region, temple, forehead, malar area, nose). In 2/3 cases the ipsilateral sclera is affected. Nevus of Ota usually remains stable once adulthood is reached but an increased risk of glaucoma and uveal melanoma may be observed. Extracutaneous lesions may also occur in cornea, retina, tympanum, nasal mucosa, pharynx, palate. Nevus of Ota occurs as solitary conditions but seldom may occur together with the nevus of Ito or nevus spilus."},"zh":{"name":"太田痣","synonyms":["眼上腭部褐青色痣"],"definition":"太田痣是一种更常见于亚洲和非洲人群的眼皮黑素细胞增多症，通常在出生时出现，其特征是通常单侧、蓝灰色、斑片状、斑点状的色素沉着(可能会逐渐扩大和变暗)，沿着三叉神经眼部和上颌部(眼周区域、太阳穴、额头、颧区和鼻子)的分布影响面部皮肤。2/3的病例累及同侧巩膜。太田痣通常在成年后保持稳定，但可能会增加青光眼和葡萄膜黑色素瘤的风险。皮肤外病灶也可能发生在角膜、视网膜、鼓室、鼻粘膜、咽、腭部。太田痣以孤立性症状出现，但很少与伊藤痣或溢痣同时出现。"},"tr":{"name":"Ota nevüsü","synonyms":["Nevüs fussuloserulös oftalmo maksillaris"]}},"xrefs":[{"source":"ICD-10","code":"D22.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LC10","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1221094078"},{"source":"MONDO","code":"0016984","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D009507","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0027961","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10051713","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":294057,"name":"Rare nevus","classifications":[187,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}