{"orpha":263432,"name":"Nevus of Ito","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Nevus fuscocaeruleus acromiodeltoideus"],"definition":"Nevus of Ito is a benign dermal melanocytosis occurring most frequently in the Asian populations and characterized by unilateral, asymptomatic, blue, gray or brown skin pigmentation within the acromioclavicular and upper chest area (involving the side of the neck, the supraclavicular and scapular areas, and the shoulder region). It is usually diagnosed in early infancy and in early adolescence. Nevus of Ito may progressively enlarge and darken in color (particularly with puberty) and its appearance usually remains stable once adulthood is reached. Spontaneous regression does not occur. Malignant melanoma has rarely been reported within a nevus of Ito. It shares the clinical features of nevus of Ota, except its anatomic location and in rare occasions, may occur together with the latter.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-263432/","orphanet_url":"https://www.orpha.net/en/disease/detail/263432","names":{"fr":{"name":"Naevus d'Ito","synonyms":["Naevus fuscocaeruleus acromiodeltoideus"],"definition":"Mélanocytose cutanée bénigne survenant le plus souvent chez les populations asiatiques et caractérisée par une pigmentation cutanée unilatérale, asymptomatique, bleue, grise ou brune dans la région acromio-claviculaire et le haut du thorax (touchant le côté du cou, les régions supraclaviculaire et scapulaire, ainsi que la région des épaules). Diagnostiqué généralement dans la petite enfance et au début de l'adolescence, le naevus d'Ito peut progressivement s'agrandir et devenir plus foncé (en particulier à la puberté), son apparence restant généralement stable à l'âge adulte. On n'observe pas de régression spontanée. Dans de rares cas, des mélanomes malins ont été observés au sein du naevus d'Ito. Il présente des caractéristiques cliniques communes avec le naevus d'Ota, à l'exception de sa localisation anatomique, et peut, dans de rares cas, apparaître en association avec celui-ci."},"de":{"name":"Naevus Ito","synonyms":["Naevus fuscocaeruleus acromiodeltoides"],"definition":"Der Naevus von Ito ist eine gutartige dermale Melanozytose, die am häufigsten in der asiatischen Bevölkerung auftritt und durch einseitige, asymptomatische, blaue, graue oder braune Hautpigmentierung im akromioklavikulären und oberen Brustbereich (inklusive der Halsseite, dem supraklavikulären und skapulären Bereichen sowie der Schulterregion) gekennzeichnet ist. Sie wird in der Regel im frühen Kindesalter und in der frühen Adoleszenz diagnostiziert. Der Nävus von Ito kann sich nach und nach vergrößern und verdunkeln (insbesondere in der Pubertät) und sein Aussehen bleibt in der Regel stabil, wenn das Erwachsenenalter erreicht ist. Eine spontane Regression findet nicht statt. In seltenen Fällen wurden maligne Melanome innerhalb des Naevus von Ito beobachtet. Es teilt die klinischen Merkmale des Nävus von Ota, mit Ausnahme seiner anatomischen Lage und in seltenen Fällen kann es zusammen mit diesem auftreten."},"es":{"name":"Nevo de Ito","synonyms":["Nevo de Ota acromiodeltoide"],"definition":"Es una melanocitosis dérmica benigna que se presenta con mayor frecuencia en poblaciones asiáticas, caracterizada por una pigmentación cutánea azulada, grisácea o parduzca asintomática, unilateral, en la zona acromioclavicular y en el tórax superior (afectando la cara lateral del cuello, la región supraclavicular y escapular, y el hombro). Suele diagnosticarse en la primera infancia y en la adolescencia temprana. Puede aumentar de tamaño y oscurecerse progresivamente (especialmente en la pubertad) permaneciendo estable una vez alcanzada la edad adulta, pero sin una regresión espontánea. Excepcionalmente se ha descrito el desarrollo de melanoma maligno sobre este tipo de nevo. Comparte características clínicas con el nevo de Ota (distinta localización anatómica) y, excepcionalmente, pueden coexistir ambas lesiones."},"it":{"name":"Nevo di Ito","synonyms":["Nevo fuscoceruleo acromiodeltoideo"],"definition":"Il nevo di Ito è una melanocitosi dermica benigna osservata prevalentemente nelle popolazioni asiatiche; è caratterizzato da pigmentazione blu, grigia o marrone, asintomatica e monolaterale, della cute all'interno della regione acromioclavicolare e della parte superiore del torace (lato del collo, regione sopraclavicolare, scapolare e spalle). Di solito viene diagnosticato nella prima infanzia e nella prima adolescenza. Il nevo di Ito si allarga e si scurisce progressivamente (soprattutto con la pubertà), per assumere un aspetto stabile una volta raggiunta l'età adulta. Non si osserva regressione spontanea. Sono stati descritti rari casi di melanoma maligno all'interno di un nevo di Ito. Presenta gli stessi segni clinici del nevo di Ota, ad eccezione della localizzazione anatomica; raramente i due nevi coesistono."},"nl":{"name":"Naevus van Ito","synonyms":["Naevus fuscocaeruleus acromiodeltoideus"],"definition":"Naevus van Ito is een goedaardige dermale melanocytose die het vaakst voorkomt in de Aziatische populaties en die gekarakteriseerd wordt door een unilaterale, asymptomatische, blauw, grijze of bruine huidpigmentatie in het acromioclaviculaire en bovenste deel van de borst (de zijkant van de hals, de supraclaviculaire en scapulaire gebieden, en het schoudergebied). Diagnose gebeurt meestal in de vroege zuigelingentijd of in de vroege adolescentie. Naevus van Ito kan progressief groter en donkerder van kleur worden (vooral in de puberteit) en eens de volwassenheid is bereikt, blijft het uiterlijk meestal stabiel. Spontane regressie komt niet voor. In zeldzame gevallen werd een kwaadaardig melanoom in een naevus van Ito gerapporteerd. De aandoening deelt de klinische kenmerken van naevus van Ota, behalve de anatomische locatie, en in zeldzame gevallen kunnen beide samen voorkomen."},"pt":{"name":"Nevus de Ito"},"pl":{"name":"Znamię Ito","synonyms":["Nevus fuscocaeruleus acromiodeltoideus"]},"cs":{"name":"Itův névus","synonyms":["Nevus fuscocaeruleus acromiodeltoideus"]},"uk":{"name":"Невус Іто","synonyms":["Невус фускокаерулеус акроміодельтоідеус (Nevus fuscocaeruleus acromiodeltoideus)"],"definition":"Nevus of Ito is a benign dermal melanocytosis occurring most frequently in the Asian populations and characterized by unilateral, asymptomatic, blue, gray or brown skin pigmentation within the acromioclavicular and upper chest area (involving the side of the neck, the supraclavicular and scapular areas, and the shoulder region). It is usually diagnosed in early infancy and in early adolescence. Nevus of Ito may progressively enlarge and darken in color (particularly with puberty) and its appearance usually remains stable once adulthood is reached. Spontaneous regression does not occur. Malignant melanoma has rarely been reported within a nevus of Ito. It shares the clinical features of nevus of Ota, except its anatomic location and in rare occasions, mayoccur together with the latter."},"zh":{"name":"伊藤痣","synonyms":["肩峰三角肌蓝褐痣"],"definition":"伊藤痣是一种最常见于亚洲人群的良性皮肤黑色素细胞增多症，其特征是肩锁和上胸区(包括颈部一侧、锁骨上区、肩胛区和肩部)的单侧、无症状、蓝色、灰色或棕色皮肤色素沉着。它通常在婴儿期和青春期早期被诊断出来。伊藤痣可能会逐渐增大和颜色变深(特别是青春期)，成年后外观通常保持稳定。不会发生自发复原。恶性黑色素瘤很少在伊藤痣中被报道。除了解剖位置以外它与太田痣具有相同的临床特征，在极少数情况下，与太田痣同时出现。"},"tr":{"name":"Ito nevüsü","synonyms":["Nevüs fussuloserulös akromiodeltoides"]}},"xrefs":[{"source":"ICD-10","code":"D22.6","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LC10","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1458402133"},{"source":"MONDO","code":"0016985","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0022283","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10830","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":"Value and class","class":"1-9 / 100 000","value":1.17,"area":"Europe","status":"Not yet validated","source":"ORPHANET"},{"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":"2024-09-30","source":"32809409[PMID]_26260725[PMID]","terms":[{"hpo":"HP:0003764","label":"Nevus","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012056","label":"Cutaneous melanoma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0025534","label":"Ocular melanocytosis","frequency":"Excluded (0%)","criteria":null}]}}