{"orpha":178517,"name":"Localized pagetoid reticulosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Pagetoid reticulosis, Woringer-Kolopp type"],"definition":"A rare variant of mycosis fungoides (MF), a form of cutaneous T-cell lymphoma, characterized by the presence of localized patches or plaques with epidermal hyperplasia and intraepidermal proliferation of neoplastic T-cells, usually involving one extremity.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-178517/","orphanet_url":"https://www.orpha.net/en/disease/detail/178517","names":{"fr":{"name":"Réticulose pagétoïde localisée","synonyms":["Réticulose pagétoïde type Woringer-Kolopp"],"definition":"Variante rare du mycosis fongoïde (MF), forme de lymphome T cutané, caractérisée par la présence de plaques ou de taches localisées, avec une hyperplasie épidermique et une prolifération intra-épidermique de cellules T néoplasiques, touchant généralement un membre."},"de":{"name":"Retikulose, lokalisierte pagetoide","synonyms":["Pagetoide Retikulose Typ Woringer-Kolopp"],"definition":"Eine seltene Variante der Mycosis fungoides (MF), einer Form des kutanen T-Zell-Lymphoms, die durch lokalisierte Flecken oder Plaques mit epidermaler Hyperplasie und intraepidermaler Proliferation neoplastischer T-Zellen gekennzeichnet ist und in der Regel eine Extremität betrifft."},"es":{"name":"Reticulosis pagetoide localizada","synonyms":["Reticulosis pagetoide tipo Woringer-Kolopp"],"definition":"Es una variante poco frecuente de micosis fungoide (MF), una forma de linfoma cutáneo de células T, caracterizada por la presencia de máculas o placas localizadas con hiperplasia epidérmica y proliferación intraepidérmica de células T neoplásicas que, por lo general, afecta a una extremidad."},"it":{"name":"Reticulosi localizzata pagetoide","definition":"La reticulosi localizzata pagetoide è una variante rara di micosi fungoide (MF; si veda questo termine), una forma di linfoma a cellule T, caratterizzata da macchie o placche localizzate associate a iperplasia epidermica e proliferazione intraepidermica delle cellule T neoplastiche, che di solito interessano un arto."},"nl":{"name":"Gelokaliseerde pagetoïde reticulose","synonyms":["Pagetoïde reticulose, Woringer-Kolopp-type"],"definition":"Een zeldzame variant van mycosis fungoides (MF), een vorm van cutaan T-cellymfoom, gekenmerkt door aanwezigheid van gelokaliseerde vlekken of plaques met epidermale hyperplasie en intra-epidermale proliferatie van neoplastische T-cellen, meestal met betrokkenheid van één extremiteit."},"pt":{"name":"Reticulose pagetóide localizada","definition":"A reticulose pagetóide localizada é uma variante rara de micose fungóide (MF; ver este termo), uma forma de linfoma cutâneo das células T, e é caracterizado pela presença de lesões eczematosas ou placas localizadas com hiperplasia epidérmica e proliferação intra-epidérmica de células T neoplásicas, envolvendo normalmente uma extremidade."},"pl":{"name":"Zlokalizowana siatkowica pagetoidalna","synonyms":["Siatkowica pagetoidalna, typu Woringera i Koloppa"]},"cs":{"name":"Lokalizovaná pagetoidní retikulóza","synonyms":["Pagetoidní retikulóza, Woringerův-Koloppův typ","Pagetoidní retikulóza, Woringer-Koloppův typ"]},"uk":{"name":"Локалізований пагетоїдний ретикулоз","synonyms":["Пагетоїдний ретикулоз, тип Ворінгера-Колоппа"],"definition":"A rare variant of mycosis fungoides (MF), a form of cutaneous T-cell lymphoma, characterized by the presence of localized patches or plaques with epidermal hyperplasia and intraepidermal proliferation of neoplastic T-cells, usually involving one extremity."},"zh":{"name":"局限性畸形性骨炎网状细胞增多症","synonyms":["Woringer-Kolopp型变形性骨炎网状细胞增多症"],"definition":"局部Paget病样网状细胞增生病是一种蕈样肉芽肿（MF）的罕见变体，是一种皮肤T细胞淋巴瘤，其特征为局部斑块、斑块伴表皮增生和表皮内T淋巴细胞增生，通常累及单侧肢体。"},"tr":{"name":"Lokalize pagetoid retiküloz","synonyms":["Pagetoid retiküloz, Woringer-Kolopp tipi"]}},"xrefs":[{"source":"ICD-10","code":"C84.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B01","qualifier":"NTBT","icd_relation":"attributed","validated":true,"icd11_uri":"null"},{"source":"MONDO","code":"0015809","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D056267","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1276140","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":178566,"name":"Mycosis fungoides and variants","classifications":[187,194,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}