{"orpha":158011,"name":"Necrobiotic xanthogranuloma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Necrobiotic xanthogranuloma is a rare, chronic and progressive, non-Langerhans cell histiocytosis disease typically characterized by multiple, indurated, asymptomatic to pruritic, yellow-orange plaques or nodules that tend to ulcerate and are usually located in the periorbital area, trunk and/or extremities. Strong association with paraproteinemia and/or malignant lymphoproliferative disease has been reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-158011/","orphanet_url":"https://www.orpha.net/en/disease/detail/158011","names":{"fr":{"name":"Xanthogranulome nécrobiotique","definition":"Maladie rare, chronique et progressive, de type histiocytose non langerhansienne, caractérisée par des plaques ou des nodules multiples, indurés, asymptomatiques à prurigineux, de couleur jaune-orange, qui tendent à s'ulcérer, localisés généralement dans la région périorbitaire, le tronc et/ou les extrémités. Une forte association avec une paraprotéinémie et/ou une maladie lymphoproliférative maligne a été rapportée."},"de":{"name":"Xanthogranulom, nekrobiotisches"},"es":{"name":"Xantogranuloma necrobiótico","definition":"Es una enfermedad de histiocitosis de células no Langerhans, progresiva, crónica y poco frecuente, caracterizada típicamente por placas o nódulos endurecidos múltiples que varían de asintomáticos a pruriginosos, de color amarillo-anaranjado, que tienden a ulcerarse y que, por lo habitual, se localizan en la zona periorbital, tronco y/o extremidades. Se ha descrito una fuerte asociación con paraproteinemia y/o neoplasias linfoproliferativas."},"it":{"name":"Xantogranuloma necrobiotico","definition":"Lo xantogranuloma necrobiotico è un'istiocitosi rara cronica e progressiva a cellule non-Langerhans, caratterizzata da placche o noduli multipli, induriti, asintomatici o pruriginosi, di colore giallo-arancione, che tendono ad ulcerarsi, di solito localizzati nella regione periorbitale, sul tronco e/o sugli arti. È stata osservata una marcata associazione con la paraproteinemia e/o con malattie linfoproliferative maligne."},"nl":{"name":"Necrobiotisch xanthogranuloom","definition":"Necrobiotisch xanthogranuloom is een zeldzame, chronische en progressieve niet-Langerhanscel histiocytose, en wordt doorgaans gekarakteriseerd door multipele, verharde, asymptomatische tot pruritische, geel-oranje plaques of noduli die de neiging hebben om te verzweren en die meestal zijn gesitueerd in de periorbitale streek, op de romp en/of op de ledematen. Er werd een sterke associatie met paraproteïnemie en/of maligne lymfoproliferatieve ziekte gerapporteerd."},"pt":{"name":"Xantogranuloma necrobiótico"},"pl":{"name":"Żółtakoziarniniakowatość z nekrobiozą","definition":"*Żółtakoziarniniakowatość z nekrobiozą jest rzadką, przewlekłą i postępującą histiocytozą z komórek innych niż Langerhansa, która typowo charakteryzuje się licznymi, stwardniałymi, bezobjawowymi lub swędzącymi, żółtopomarańczowymi płytkami lub guzkami, które mają tendencję do wrzodzenia i zwykle lokalizują się w okolicy okołooczodołowej, na tułowiu i/lub kończynach. Opisywano silne powiązanie z paraproteinemią i/lub złośliwą chorobą limfoproliferacyjną."},"cs":{"name":"Nekrobiotický xantogranulom"},"uk":{"name":"Некробіотична ксантогранульома","definition":"Necrobiotic xanthogranuloma is a rare, chronic and progressive, non-Langerhans cell histiocytosis disease typically characterized by multiple, indurated, asymptomatic to pruritic, yellow-orange plaques or nodules that tend to ulcerate and are usually located in the periorbital area, trunk and/or extremities. Strong association with paraproteinemia and/or malignant lymphoproliferative disease has been reported."},"zh":{"name":"渐进性坏死性黄肉芽肿","definition":"渐进性坏死性黄肉芽肿是一种罕见的慢性进行性非朗格汉斯细胞组织细胞增生症，其典型特征为多发性、硬化性、无症状至瘙痒、黄橙色斑块或结节，易溃烂，通常位于眼眶周围、躯干和/或四肢。据报道，该病与副蛋白血症和/或恶性淋巴增生性疾病有很强的相关性。"},"tr":{"name":"Nekrobiyotik ksantogranülom"}},"xrefs":[{"source":"ICD-10","code":"D76.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EE8Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"2138563851"},{"source":"MONDO","code":"0015537","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D058252","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1275339","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10951","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":157987,"name":"Non-Langerhans cell histiocytosis","classifications":[187,196]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}