{"orpha":178512,"name":"Folliculotropic mycosis fungoides","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Mycosis fungoides-associated follicular mucinosis"],"definition":"A variant of mycosis fungoides (MF) characterized by the presence of folliculotropic infiltrates, often with sparing of the epidermis and preferential involvement of the head and neck region. Most cases show mucinous degeneration of the hair follicles (follicular mucinosis) and are traditionally designated as MF-associated follicular mucinosis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-178512/","orphanet_url":"https://www.orpha.net/en/disease/detail/178512","names":{"fr":{"name":"Mycosis fongoïde folliculotrope","synonyms":["Mycosis fongoïde avec mucinose folliculaire"],"definition":"Variante du mycosis fongoïde (MF) caractérisée par la présence d'infiltrats folliculotropes, épargnant souvent l'épiderme et touchant principalement la région de la tête et du cou. Une dégénérescence mucineuse des follicules pileux (mucinose folliculaire) est présente dans la plupart des cas traditionnellement appelés mucinose folliculaire associée au MF."},"de":{"name":"Mycosis fungoides, follikulotrope","synonyms":["Muzinose, follikuläre, Mycosis fungoides-assoziierte"],"definition":"Eine Variante der Mycosis fungoides (MF), die durch das Vorhandensein follikulotropischer Infiltrate gekennzeichnet ist, wobei häufig die Epidermis verschont bleibt und vorzugsweise der Kopf- und Halsbereich betroffen ist. Die meisten Fälle weisen eine muzinöse Degeneration der Haarfollikel (follikuläre Muzinose) auf und werden traditionell als MF-assoziierte follikuläre Muzinose bezeichnet."},"es":{"name":"Micosis fungoide foliculotropa","synonyms":["Mucinosis folicular asociada a micosis fungoide"],"definition":"Es una variante de micosis fungoide (MF) caracterizada por la presencia de infiltrados foliculotropos, a menudo respetando la epidermis y con una afectación preferente de la región de la cabeza y el cuello. En la mayoría de los pacientes se evidencia una degeneración mucinosa de los folículos pilosos (mucinosis folicular) conocida tradicionalmente como mucinosis folicular asociada a MF."},"it":{"name":"Micosi fungoide follicolotropica","definition":"La micosi fungoide follicolotropica è una variante rara della micosi fungoide (MF; si veda questo termine), una forma di linfoma cutaneo a cellule T, caratterizzata da infiltrati follicolotropici nelle lesioni che sono simili a macchie o a placche e si localizzano sulla testa e sul collo."},"nl":{"name":"Folliculotrope mycosis fungoides","synonyms":["Mycosis fungoides-geassocieerde folliculaire mucinose"],"definition":"Een variant van mycosis fungoides (MF), gekenmerkt door de aanwezigheid van folliculotrope infiltraten, waarbij de epidermis vaak gespaard blijft en vooral de regio van het hoofd en de hals/nek betrokken is. De meeste gevallen tonen mucineuze degeneratie van haarfollikels (folliculaire mucinose) en worden doorgaans aangeduid met MF-geassocieerde folliculaire mucinose."},"pt":{"name":"Micose fungóide foliculotrópica","definition":"A micose fungóide foliculotrópica é uma variante rara de micose fungóide (MF; ver este termo), uma forma de linfoma cutâneo das células T, e é caracterizada pela presença de infiltrados foliculotrópicos máculas ou placas eczematosas envolvendo normalmente a cabeça e zona do pescoço."},"pl":{"name":"Ziarniniak grzybiasty folikulotropowy","synonyms":["Ziarniniak grzybiasty z towarzyszącą mucynozą pęcherzykową"]},"cs":{"name":"Mycosis fungoides - folikulotropní varianta","synonyms":["Mycosis fungoides asociovaná s folikulární mucinózou"]},"uk":{"name":"Фолікулотропний мікоз фунгоїдів","synonyms":["Фолікулярний муциноз, асоційований з мікозом фунгоїдів"],"definition":"A rare variant of mycosis fungoides (MF), a form of cutaneous T-cell lymphoma, characterized by the presence of folliculotropic infiltrates in patch-plaque lesions usually involving the head and neck area."},"zh":{"name":"亲毛囊性蕈样肉芽肿","synonyms":["蕈样肉芽肿相关毛囊皮脂腺粘蛋白累积病"],"definition":"滤泡性蕈样肉芽肿是一种蕈样肉芽肿（MF）的罕见变体，是一种皮肤T细胞淋巴瘤，其特征是为头颈部斑块状病变存在滤泡浸润。"},"tr":{"name":"Folikülotropik mikozis fungoides","synonyms":["Mikoz fungoides-ilişkili foliküler müsinoz"]}},"xrefs":[{"source":"ICD-10","code":"C84.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B01","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1335995469"},{"source":"MONDO","code":"0015808","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1627767","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}