{"orpha":178528,"name":"Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Berti lymphoma","Primary cutaneous epidermotropic cytotoxic CD8+ T-cell lymphoma"],"definition":"Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma is a rare form of primary cutaneous T-cell lymphoma characterized by rapidly progressing, localized or disseminated nodules, tumors or eczematous skin lesions. It has a particularly aggressive clinical course with a high tendency to spread, in advanced stages, to extracutaneous locations (the central nervous system, lung, testes). Lymph nodes are often spared.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-178528/","orphanet_url":"https://www.orpha.net/en/disease/detail/178528","names":{"fr":{"name":"Lymphome T cutané épidermotrope CD8+ d'évolution agressive","synonyms":["Lymphome agressif épidermotrope type Berti"],"definition":"Le lymphome T cutané épidermotrope CD8+ d'évolution agressive est une forme rare de lymphome T cutané primaire caractérisé par des nodules, des tumeurs ou des lésions cutanées eczémateuses localisées ou disséminées, progressant rapidement. Son évolution clinique est particulièrement agressive, et le lymphome a une forte tendance à s'étendre à des sites extra-cutanés dans les stades avancés de la maladie (système nerveux central, poumons, testicules). Les ganglions lymphatiques sont souvent épargnés."},"de":{"name":"T-Zell-Lymphom, primär kutanes, aggressives epidermotropes, CD8+","synonyms":["Berti-Lymphom","Primäres kutanes epidermotrophes zytotoxisches CD8+ T-Zell-Lymphom"],"definition":"Das primäre kutane aggressive epidermotrope CD8+ T-Zell-Lymphom ist eine seltene Form des primären kutanen T-Zell-Lymphoms, das durch schnell fortschreitende, lokalisierte oder disseminierte Knötchen, Tumore oder ekzematöse Hautläsionen gekennzeichnet ist. Es hat einen besonders aggressiven klinischen Verlauf mit einer hohen Tendenz zur Ausbreitung in fortgeschrittenen Stadien auf extrakutane Lokalisationen (zentrales Nervensystem, Lunge, Hoden). Lymphknoten werden oft verschont."},"es":{"name":"Linfoma primario cutáneo agresivo epidermotrópico de células T CD8+","synonyms":["Linfoma de Berti","Linfoma primario cutáneo epidermotrópico de células T citotóxicas CD8+"],"definition":"El linfoma cutáneo primario agresivo epidermotrópico de células T CD8+ es una forma poco frecuente de linfoma cutáneo primario de células T caracterizado por nódulos, tumores o lesiones cutáneas eczematosas que progresan rápidamente, localizados o diseminados. Presenta un curso clínico particularmente agresivo con una elevada tendencia a expandirse en estadios más avanzados a localizaciones extracutáneas (sistema nervioso central, pulmones, testículos). En muchos casos, los nódulos linfáticos no estan afectados."},"it":{"name":"Linfoma cutaneo primitivo a cellule T CD8+ epidermotropo aggressivo","synonyms":["Linfoma cutaneo primitivo a cellule T CD8+ epidermotropo citotossico","Linfoma di Berti"],"definition":"Il linfoma cutaneo primitivo a cellule T CD8+ epidermotropo aggressivo è una forma rara di linfoma cutaneo primitivo a cellule T; è caratterizzato dalla presenza di noduli, tumori o lesioni cutanee eczematose a progressione rapida, localizzati o disseminati. Il decorso clinico è molto aggressivo, con una marcata tendenza alla disseminazione, negli stadi avanzati, a sedi extracutanee (sistema nervoso centrale, polmoni e testicoli). L'interessamento dei linfonodi è raro."},"nl":{"name":"Primair cutaan agressief epidermotroop CD8+ T-cellymfoom","synonyms":["Bertilymfoom","Primair cutaan epidermotroop cytotoxisch CD8+ T-cellymfoom"],"definition":"Primair cutaan agressief epidermotroop CD8+ T-cellymfoom is een zeldzame vorm van primair cutaan T-cellymfoom die gekarakteriseerd wordt door snel progressieve, gelokaliseerde of uitgezaaide noduli, tumoren of eczemateuze huidletsels. De ziekte heeft een zeer agressief klinisch verloop met, in gevorderde stadia, een sterke neiging tot uitzaaiing naar extracutane locaties (het centrale zenuwstelsel, de longen, de testes). Lymfeklieren blijven vaak gespaard."},"pt":{"name":"Linfoma primário cutâneo epidermotrópico agressivo de celúlas T CD8+"},"pl":{"name":"Pierwotny skórny chłoniak agresywny epidermotropowy z komórek T CD8+","synonyms":["Chłoniak Bertiego","Pierwotny skórny chłoniak epidermotropowy cytotoksyczny z komórek T CD8+"]},"cs":{"name":"Primární kožní agresivní epidermotropní CD8+ T-buněčný lymfom","synonyms":["Bertiho lymfom","Primární kožní epidermotropní cytotoxický CD8+ T-buněčný lymfom"]},"uk":{"name":"Первинна шкірна агресивна епідермотропна CD8+ Т-клітинна лімфома","synonyms":["Лімфома Берті","Первинна шкірна епідермотропна цитотоксична CD8+ Т-клітинна лімфома"],"definition":"Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma is a rare form of primary cutaneous T-cell lymphoma characterized by rapidly progressing, localized or disseminated nodules, tumors or eczematous skin lesions. It has a particularly aggressive clinical course with a high tendency to spread, in advanced stages, to extracutaneous locations (the central nervous system, lung, testes). Lymph nodes are often spared."},"zh":{"name":"原发性皮肤侵袭性亲表皮CD8阳性细胞毒性T细胞淋巴瘤","synonyms":["原发性皮肤侵袭性亲表皮CD8阳性细胞毒性T细胞淋巴瘤"],"definition":"原发性皮肤侵袭性表皮性CD8+T细胞淋巴瘤是一种罕见的原发性皮肤T细胞淋巴瘤，其主要临床表现为快速进展的局限性或散播性结节、肿瘤或湿疹性皮肤病变。该病侵袭性强，晚期蔓延到皮肤外组织（中枢神经系统，肺，睾丸），通常不累及淋巴结。"},"tr":{"name":"Primer kutanöz agresif epidermotropik CD8 + T hücreli lenfoma","synonyms":["Berti lenfoma","Primer kutanöz epidermotropik sitotoksik CD8 + T hücreli lenfoma"]}},"xrefs":[{"source":"ICD-10","code":"C84.5","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B0Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1228062926"},{"source":"MONDO","code":"0015811","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4518232","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":178551,"name":"Aggressive primary cutaneous T-cell lymphoma","classifications":[187,194,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}