{"orpha":300504,"name":"Onychocytic matricoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Acanthoma of the nail matrix"],"definition":"A rare, benign, nail tumor originating in the nail matrix characterized by localized pachyonychia and variable degrees of pigmentation: pigmented, melanocytic (common, longitudinal melanonychia that may simulate a foreign body) or hypopigmented. Histopathology demonstrates a purely epithelial tumor with endokeratinization in the deep portion and concentrically arranged nests of prekeratogenous and keratogenous cells.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-300504/","orphanet_url":"https://www.orpha.net/en/disease/detail/300504","names":{"fr":{"name":"Matricome onychocytique","synonyms":["Acanthome de la matrice des ongles","Acanthome de la matrice unguéale"],"definition":"Tumeur rare et bénigne des ongles prenant naissance dans la matrice de l'ongle, caractérisée par une pachyonychie localisée et des lésions de pigmentation de degré variable&#8239;: lésions pigmentées, lésions de pigmentation mélanocytaire (mélanonychie longitudinale fréquente pouvant ressembler à un corps étranger) ou lésions hypopigmentées. L'examen histopathologique met en évidence une tumeur purement épithéliale avec une endokératinisation dans la partie profonde et des foyers de cellules prékératogènes et kératogènes disposés de manière concentrique."},"de":{"name":"Onychozytisches Matrikom","synonyms":["Akanthom der Nagelmatrix"]},"es":{"name":"Matricoma onicocítico","synonyms":["Acantoma de la matriz ungueal"],"definition":"Es un tumor ungueal poco frecuente y benigno que se origina en la matriz ungueal y que está caracterizado por paquioniquia localizada y por diferentes grados de pigmentación: pigmentada, melanocítica (común; melanoniquia longitudinal, que puede simular a un cuerpo extraño) o hipopigmentada. La histopatología evidencia un tumor epitelial puro con endoqueratinización en la capa profunda y nidos concéntricos de células prequeratógenas y queratógenas."},"it":{"name":"Matricoma onicocitico","synonyms":["Acantoma della matrice ungueale"],"definition":"Il matricoma onicocitico è una neoplasia ungueale benigna rara, che origina dalla matrice dell'unghia ed è caratterizzata da pachionichia localizzata e pigmentazione variabile: può essere pigmentata, melanocitica (la forma comune, caratterizzata da melanonichia longitudinale che ricorda la forma di un corpo estraneo), oppure ipopigmentata. Gli esami istopatologici evidenziano un tumore esclusivamente epiteliale con endocheratinizzazione della porzione profonda e nidi di cellule precheratogene e cheratogene disposti in maniera concentrica."},"nl":{"name":"Onychocytisch matricoom","synonyms":["Acanthoom van nagelmatrix"],"definition":"Onychocytisch matricoom is een zeldzame, goedaardige nageltumor die ontstaat in de nagelmatrix en die gekarakteriseerd wordt door gelokaliseerde pachyonychia en variabele gradaties van pigmentatie: gepigmenteerd, melanocytisch (gangbare, longitudinale melanonychia die een vreemd lichaam kan simuleren) of gehypopigmenteerd. Histopathologie toont een zuiver epitheliale tumor met endokeratinisatie in de diepe delen en concentrisch geschikte nesten met prekeratogene en keratogene cellen."},"pt":{"name":"Matricoma onicocítico"},"pl":{"name":"Rogowiak macierzy paznokcia","synonyms":["Rogowiak macierzy paznokcia","Akantoza macierzy paznokcia"]},"cs":{"name":"Onychocytární matrikom","synonyms":["Akantom matrix nehtů"]},"uk":{"name":"Оніхоцитарна матрикома","synonyms":["Акантома нігтьового матриксу"],"definition":"A rare, benign, nail tumor originating in the nail matrix characterized by localized pachyonychia and variable degrees of pigmentation: pigmented, melanocytic (common, longitudinal melanonychia that may simulate a foreign body) or hypopigmented. Histopathology demonstrates a purely epithelial tumor with endokeratinization in the deep portion and concentrically arranged nests of prekeratogenous and keratogenous cells."},"zh":{"name":"甲细胞间质瘤","synonyms":["甲基质棘皮瘤"],"definition":"甲细胞母质瘤是一种罕见的良性指甲肿瘤，起源于指甲基质，其特征是局灶性指甲肥厚和不同程度色素沉着：色素性黑素细胞（常见的，纵行黑甲可模拟异物）或色素减退。病理组织学显示一个单纯上皮肿瘤，深部有内角化，角化前细胞和角化细胞呈巢状排列。"},"tr":{"name":"Onikositik matrikom","synonyms":["Tırnak matrisinin akantoması"]}},"xrefs":[{"source":"ICD-10","code":"D23.9","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0017586","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4751108","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"11007","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":300515,"name":"Rare nail tumor","classifications":[187,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adolescent","Adult","Childhood","Elderly"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":5.0,"area":"Worldwide","status":"Validated","source":"22197859[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}