{"orpha":300512,"name":"Onychomatricoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Onychomatricoma is a rare, benign nail tumor originating in the nail matrix characterized by localized or diffuse thickening of the nail plate, increased transverse or longitudinal overcurvature, a yellow longitudinal band of variable width, swelling of the proximal nail fold, multiple splinter hemorrhages and the presence of honeycomb-like cavities in the distal margin of the nail plate. Nail dystrophy and dorsal pterygium may be associated. Occasionally, a pigmented lesion has been reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-300512/","orphanet_url":"https://www.orpha.net/en/disease/detail/300512","names":{"fr":{"name":"Onychomatricome","definition":"Tumeur rare et bénigne des ongles prenant naissance dans la matrice de l'ongle, caractérisée par un épaississement localisé ou diffus de la tablette unguéale, une courbure transversale ou longitudinale excessive, une bande jaune longitudinale de largeur variable, un gonflement du pli proximal de l'ongle, de multiples hémorragies en flammèches et la présence de cavités en rayons de miel dans la région distale de la tablette unguéale. Une dystrophie des ongles et un pterygium dorsal peuvent être associés. Dans certains cas, une lésion pigmentée a été rapportée."},"de":{"name":"Onychomatrikom"},"es":{"name":"Onicomatricoma","definition":"Es un tumor de las uñas benigno y poco frecuente que se origina en la matriz ungueal. Se caracteriza por engrosamiento difuso o localizado de la placa ungueal, aumento transversal o longitudinal de la curvatura, presencia de una banda amarilla longitudinal de anchura variable, edema del pliegue proximal de la uña, múltiples hemorragias en astilla y presencia de cavidades ''en panal'' en el margen distal de la placa ungueal. También pueden estar asociados distrofia ungueal y pterigión dorsal. Ocasionalmente se ha descrito la presencia de lesiones pigmentadas."},"it":{"name":"Onicomatricoma","definition":"L'onicomatricoma è un tumore benigno raro delle unghie, che origina dalla matrice ungueale. È caratterizzato da un ispessimento localizzato o diffuso della lamina dell'unghia, aumento della curvatura trasversale o longitudinale, presenza di una striscia longitudinale di colore giallo e di larghezza variabile, tumefazione della piega ungueale prossimale, emorragie a scheggia multiple e presenza di una cavità a nido d'ape nel margine distale della lamina dell'unghia. Può associarsi a distrofia dell'unghia e pterigio dorsale. Sono state segnalate occasionali lesioni pigmentate."},"nl":{"name":"Onychomatricoom","definition":"Onychomatricoom is een zeldzame, goedaardige nageltumor die ontstaat in de nagelmatrix en die gekarakteriseerd wordt door gelokaliseerde of diffuse verdikking van de nagelplaat, toegenomen transversale of longitudinale overdreven kromming, een gele longitudinale band met variabele breedte, zwelling van de proximale nagelplooi, multipele splinterbloedingen, en de aanwezigheid van honingraatachtige holtes in de distale rand van de nagelplaat. Nageldystrofie en dorsaal pterygium kunnen geassocieerd zijn. Occasioneel werd een gepigmenteerd letsel gerapporteerd."},"pt":{"name":"Onicomatricoma"},"pl":{"name":"Guz macierzy paznokcia","definition":"*Guz macierzy paznokcia jest rzadkim, łagodnym guzem paznokcia, który wywodzi się z macierzy paznokcia i charakteryzuje miejscowym lub rozlanym zgrubieniem płytki paznokcia, zwiększoną liczbą poprzecznych lub podłużnych bruzd, żółtym podłużnym pasmem o zmiennej szerokości, obrzękiem proksymalnego fałdu paznokcia, licznymi linijnymi krwotokami podpaznokciowymi i obecnością ubytków przypominających plaster miodu na dystalnym brzegu płytki paznokcia. Może towarzyszyć dystrofia paznokci i skrzydlik grzbietowy paznokcia. Sporadycznie opisywano zmiany barwnikowe."},"cs":{"name":"Onychomatrikom"},"uk":{"name":"Оніхоматрикома","definition":"Onychomatricoma is a rare, benign nail tumor originating in the nail matrix characterized by localized or diffuse thickening of the nail plate, increased transverse or longitudinal overcurvature, a yellow longitudinal band of variable width, swelling of the proximal nail fold, multiple splinter hemorrhages and the presence of honeycomb-like cavities in the distal margin of the nail plate. Nail dystrophy and dorsal pterygium may be associated. Occasionally, a pigmented lesion has been reported."},"zh":{"name":"甲膜瘤","definition":"甲基质瘤是一种罕见的良性指甲肿瘤，起源于指甲基质，其特征是甲盖局部或弥漫性增厚，横向或纵向过度弯曲，宽度可变的黄色纵向带，近端甲襞肿胀，多发性碎片出血，甲板远端边缘蜂窝状腔。指甲营养不良可能与背侧翼状胬肉有关。偶有色素性病变的报道。"},"tr":{"name":"Onikomatrikom"}},"xrefs":[{"source":"ICD-10","code":"D23.9","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0017587","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4751078","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":["Unknown"],"onset":["All ages"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":50.0,"area":"Worldwide","status":"Validated","source":"21965578[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}