{"orpha":180275,"name":"Paget disease of the nipple","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Mammary Paget disease","Paget disease of the breast","Paget's disease of the nipple"],"definition":"Paget disease of the nipple describes a rare presentation of breast cancer, seen most frequently in women aged 50-60, manifesting with nipple drainage and itching, erythema, crusty and excoriated nipple, thickened plaques, and hyperpigmentation (less frequently). It is due to tumor cells invading the nipple-areola complex and represents 1-3% of all new breast cancer diagnoses.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-180275/","orphanet_url":"https://www.orpha.net/en/disease/detail/180275","names":{"fr":{"name":"Maladie de Paget du mamelon","synonyms":["Maladie de Paget du sein","Maladie de Paget mammaire"],"definition":"La maladie de Paget du mamelon décrit une présentation rare du cancer du sein, observée le plus souvent chez les femmes âgées de 50-60 ans. Elle se manifeste par un drainage et une démangeaison au niveau du mamelon, un érythème, un mamelon croûteux et excorié, des plaques épaisses et une hyperpigmentation (moins fréquemment). Elle est due à l'invasion du complexe auréole-mamelon par des cellules tumorales et représente 1-3% de l'ensemble des nouveaux diagnostics de cancer mammaire."},"de":{"name":"Paget-Krankheit der Brustwarze","synonyms":["Paget-Karzinom der Brustwarze","Paget-Krankheit der Mamille"]},"es":{"name":"Enfermedad de Paget del pezón","synonyms":["Enfermedad de Paget del pecho","Enfermedad de Paget mamaria"],"definition":"La enfermedad de Paget del pezón describe una presentación rara del cáncer de mama, visto con mayor frecuencia en mujeres de 50-60 años de edad, que se manifiesta con secreción del pezón y picazón, eritema, costras, pezón excoriado, placas engrosadas e hiperpigmentación (con menos frecuencia). Es debida a la presencia de células tumorales que invaden el complejo areola-pezón y representa entre 1-3% de todos los cánceres de mama diagnosticados."},"it":{"name":"Malattia del capezzolo di Paget","definition":"La malattia del capezzolo di Paget è un cancro raro della mammella, che si osserva soprattutto nelle donne di 50-60 anni, e si manifesta con secrezioni e prurito a livello del capezzolo, che appare eritematoso, ricoperto di croste ed escoriazioni, con placche ispessite e iperpigmentazione (più raramente). È dovuta a cellule tumorali, che invadono il complesso capezzolo-areola. Rappresenta l'1-3% di tutte le nuove diagnosi di cancro della mammella."},"nl":{"name":"Ziekte van Paget van tepel","synonyms":["Mammaire ziekte van Paget","Ziekte van Paget van borst"],"definition":"Ziekte van Paget van de tepel beschrijft een zeldzame vorm van borstkanker die voornamelijk wordt aangetroffen bij vrouwen tussen 50-60 jaar en zich manifesteert met vochtafscheiding uit en jeuk aan de tepel, erytheem, korstvorming en ontvelling aan de tepel, verdikte plaques en hyperpigmentatie (minder voorkomend). De ziekte wordt veroorzaakt door tumorcellen die binnendringen in het tepel-areola complex en vertegenwoordigt 1-3% van alle nieuwe diagnoses met borstkanker."},"pt":{"name":"Doença de Paget do mamilo"},"pl":{"name":"Choroba Pageta sutka","synonyms":["Mammary Paget disease","Paget disease of the breast","Paget's disease of the nipple"]},"cs":{"name":"Pagetova choroba bradavky","synonyms":["Mammary Paget disease","Paget disease of the breast","Paget's disease of the nipple"]},"uk":{"name":"Хвороба Педжета соска","synonyms":["Хвороба молочної залози Педжета","Хвороба Педжета молочної залози","Хвороба Педжета соска"],"definition":"Paget disease of the nipple describes a rare presentation of breast cancer, seen most frequently in women aged 50-60, manifesting with nipple drainage and itching, erythema, crusty and excoriated nipple, thickened plaques, and hyperpigmentation (less frequently). It is due to tumor cells invading the nipple-areola complex and represents 1-3% of all new breast cancer diagnoses."},"zh":{"name":"乳头Paget病","synonyms":["乳头佩吉特病"],"definition":"乳头Paget病是一种罕见的乳腺癌，最常发生于50-60岁的女性，其主要临床表现为乳头表面渗液、瘙痒，有红斑、结痂，乳头破坏甚至脱落、斑块增厚和色素沉着（不太常见）。由于肿瘤细胞侵入乳头乳晕复合体所致，占所有新诊断乳腺癌的1-3%。"},"tr":{"name":"Meme ucunun paget hastalığı","synonyms":["Memeli Paget hastalığı","Memenin Paget hastalığı","Meme ucunun Paget hastalığı"]}},"xrefs":[{"source":"ICD-10","code":"C50.0","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-11","code":"2E65.5","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1295910447"},{"source":"MONDO","code":"0015873","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D010144","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1704323","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"7303","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10033364","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":180257,"name":"Rare malignant breast tumor","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.51,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.065,"area":"Finland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.096,"area":"Poland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.179,"area":"Austria","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.433,"area":"Belgium","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.235,"area":"Bulgaria","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.192,"area":"Croatia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.287,"area":"Czech Republic","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.434,"area":"Estonia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.29,"area":"Germany","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.682,"area":"Iceland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.487,"area":"Ireland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.421,"area":"Italy","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.254,"area":"Latvia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.215,"area":"Lithuania","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.345,"area":"Malta","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.709,"area":"Norway","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.209,"area":"Portugal","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.21,"area":"Slovakia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.457,"area":"Slovenia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.415,"area":"Spain","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.394,"area":"Switzerland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.335,"area":"Netherlands","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.637,"area":"United Kingdom","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}