{"orpha":178566,"name":"Mycosis fungoides and variants","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":[],"definition":"A group of cutaneous T-cell lymphomas characterized by the indolent, slow progression of patches, plaques, and tumors. There are four known variants: classic mycosis fungoides, folliculotropic mycosis fungoides, localized pagetoid reticulosis and granulomatous slack skin. Classic MF is characterized by slow progression from patches on sun-protracted areas to more infiltrated plaques and tumors; folliculotropic MF preferentially involves the head and neck region; localized pagetoid reticulosis shows localized patches or plaques, usually involving one extremity; granulomatous slack skin is extremely rare and characterized by the presence of circumscribed areas of pendulous lax skin.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-178566/","orphanet_url":"https://www.orpha.net/en/disease/detail/178566","names":{"fr":{"name":"Mycosis fongoïde et variants","definition":"Groupe de lymphomes cutanés à lymphocytes T caractérisés par une progression indolente et lente de plaques, de taches et de tumeurs. Il existe quatre variantes connues : la mycosis fongoïde classique, la mycosis fongoïde folliculotrope, la réticulose pagétoïde localisée et la peau flasque granulomateuse. La mycosis fongoïde classique se caractérise par une progression lente, passant de plaques sur les zones exposées au soleil à des plaques et à des tumeurs plus infiltrantes ; la mycosis fongoïde folliculotrope touche principalement la région de la tête et du cou ; la réticulose pagétoïde localisée se manifeste par des plaques localisées, touchant généralement un membre ; la peau flasque granulomateuse est extrêmement rare et se caractérise par la présence de zones circonscrites de peau flasque et lâche."},"de":{"name":"Mycosis fungoides-Varianten","definition":"Eine Gruppe von kutanen T-Zell-Lymphomen, die durch ein indolentes, langsames Fortschreiten von Flecken, Plaques und Tumoren gekennzeichnet sind. Es sind vier Varianten bekannt: klassische Mycosis fungoides (MF), follikulotrope MF, lokalisierte pagetoidale Retikulose und granulomatöse schlaffe Haut. Die klassische MF ist durch ein langsames Fortschreiten von Flecken an sonnenexponierten Stellen zu stärker infiltrierten Plaques und Tumoren gekennzeichnet; die follikulotrope MF betrifft vorzugsweise den Kopf- und Halsbereich; die lokalisierte pagetoidale Retikulose zeigt lokalisierte Flecken oder Plaques, die in der Regel eine Extremität betreffen; die granulomatöse schlaffe Haut ist äußerst selten und durch das Vorhandensein umschriebener Bereiche mit hängender, schlaffer Haut gekennzeichnet."},"es":{"name":"Micosis fungoide y variantes","definition":"Es un grupo de linfomas cutáneos de células T caracterizados por la progresión lenta e indolente de máculas, placas y tumores. Existen cuatro variantes conocidas: micosis fungoide clásica, micosis fungoide foliculotrópica, reticulosis pagetoide localizada y piel flácida granulomatosa. La MF clásica se caracteriza por una progresión lenta desde máculas en zonas de exposición prolongada al sol hasta placas y tumores más infiltrados; la MF foliculotrópica afecta preferentemente a la región de cabeza y del cuello; la reticulosis pagetoide localizada muestra máculas o placas localizadas, que suelen afectar a una extremidad; la piel flácida granulomatosa es extremadamente infrecuente y se caracteriza por la presencia de áreas circunscritas de piel flácida y colgante."},"it":{"name":"Micosi fungoide e varianti"},"nl":{"name":"Mycosis fungoides en varianten","definition":"Een groep van cutane T-cellymfomen, gekenmerkt door de indolente, trage progressie van vlekken, plaques, en tumoren. Er zijn vier varianten gekend: klassieke mycosis fungoides, folliculotrope mycosis fungoides, gelokaliseerde pagetoïde reticulose en granulomateuze slappe huid. Klassieke MF wordt gekenmerkt door trage progressie van vlekken op aan de zon blootgestelde gebieden tot meer geïnfiltreerde plaques en tumoren; folliculotrope MF treft vooral het hoofd- en halsgebied; gelokaliseerde pagetoïde reticulose toont gelokaliseerde vlekken en plaques, meestal met betrokkenheid van één extremiteit; granulomateuze slappe huid is uiterst zeldzaam en wordt gekenmerkt door de aanwezigheid van begrensde gebieden van hangende slappe huid."},"pt":{"name":"Micose fungóide e variantes","definition":"A micose fungóide (MF) e as suas variantes representam as formas mais comuns de linfoma cutâneo de células T. O termo MF é restrito à forma clássica caracterizada pela progressão lenta de lesões eczematosas, placas e tumores, e para variantes com um curso indolente semelhante."},"pl":{"name":"Ziarniniak grzybiasty i warianty"},"cs":{"name":"Mycosis fungoides a klinické varianty"},"uk":{"name":"Фунгоїдні мікози та їх різновиди","definition":"A group of disorders including the most common forms of cutaneous T-cell lymphomas. The term Mycosis fungoides (MF) is restricted to the classical form characterized by the slow progression of patches, plaques and tumors, and to variants with a similar indolent course."},"zh":{"name":"蕈样真菌病及其变种","definition":"蕈样肉芽肿和变异体是一组疾病，包括最常见的皮肤T细胞淋巴瘤。蕈样肉芽肿（MF）一词仅限于经典的缓慢进展的斑块和肿瘤以及惰性变异体。"},"tr":{"name":"Mikoz fungoides ve çeşitleri"}},"xrefs":[{"source":"ICD-10","code":"C84.0","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"2B01","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"2036068731"},{"source":"MONDO","code":"0015821","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"15821","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1513782","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":178548,"name":"Indolent primary cutaneous T-cell lymphoma","classifications":[187,194,202,233]}],"children":[{"orpha":2584,"name":"Classic mycosis fungoides","type":"Disease"},{"orpha":178512,"name":"Folliculotropic mycosis fungoides","type":"Disease"},{"orpha":33111,"name":"Granulomatous slack skin","type":"Disease"},{"orpha":178517,"name":"Localized pagetoid reticulosis","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.59,"area":"Europe","status":"Validated","source":"ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}