{"orpha":2584,"name":"Classic mycosis fungoides","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Mycosis fungoides, Alibert-Bazin type"],"definition":"A form of cutaneous T-cell lymphoma characterized by slow progression from patches to more infiltrated plaques and eventually to tumors. In classic mycosis fungoides (MF), extracutaneous involvement may occur.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-2584/","orphanet_url":"https://www.orpha.net/en/disease/detail/2584","names":{"fr":{"name":"Mycosis fongoïde classique","synonyms":["Mycosis fongoïde type Alibert-Bazin"],"definition":"Forme de lymphome T cutané caractérisé par une progression lente, passant de taches à des plaques plus infiltrées, puis finalement à des tumeurs. Dans le cas d'un mycosis fongoïde classique (MF), une atteinte extracutanée est possible."},"de":{"name":"Klassische Mycosis fungoides","synonyms":["Mycosis fungoides Typ Alibert-Bazin"],"definition":"Eine Form des kutanen T-Zell-Lymphoms, die durch ein langsames Fortschreiten von Flecken zu stärker infiltrierten Plaques und schließlich zu Tumoren gekennzeichnet ist. Bei der klassischen Mycosis fungoides (MF) kann es zu einer extrakutanen Beteiligung kommen."},"es":{"name":"Micosis fungoide clásica","synonyms":["Micosis fungoide tipo Alibert-Bazin"],"definition":"Es una forma de linfoma cutáneo de células T caracterizado por una progresión lenta desde máculas a placas infiltradas y, finalmente, a tumores. La micosis fungoide (MF) clásica, puede presentar afectación extracutánea."},"it":{"name":"Micosi fungoide classica","synonyms":["Micosi fungoide tipo Alibert-Bazin"]},"nl":{"name":"Klassieke mycosis fungoides","synonyms":["Mycosis fungoides, Alibert-Bazin-type"],"definition":"Een vorm van cutaan T-cellymfoom, gekenmerkt door trage progressie van huidvlekken tot meer geïnfiltreerde plaques en uiteindelijk tot tumoren. Bij klassieke mycosis fungoides (MF) kan extracutane betrokkenheid optreden."},"pt":{"name":"Micose fungoide clássica","synonyms":["Micose fungoide, tipo Alibert-Bazin"],"definition":"A micose fungóide clássica é o tipo mais comum de micose fungóide (MF; ver este termo), é uma forma de linfoma cutâneo de células T, e é caracterizado por progressão lenta desde lesões eczematosas para placas mais infiltradas e eventualmente tumores."},"pl":{"name":"Klasyczny ziarniniak grzybiasty","synonyms":["Ziarniniak grzybiasty typu Aliberta i Bazina"]},"cs":{"name":"Klasická forma mycosis fungoides","synonyms":["Mycosis fungoides, typ Alibert-Bazin"]},"uk":{"name":"Класичні грибкові мікози","synonyms":["Мікоз фунгоїдний, тип Аліберта-Базена"],"definition":"Classical mycosis fungoides is the most common type of mycosis fungoides (MF; see this term), a form of cutaneous T-cell lymphoma, and is characterized by slow progression from patches to more infiltrated plaques and eventually to tumors."},"zh":{"name":"经典型蕈样真菌病","synonyms":["蕈样肉芽肿病，Alibert-Bazin亚型"]},"tr":{"name":"Klasik mikoz fungoides","synonyms":["Mikoz fungoides, Alibert-Bazin tipi"]}},"xrefs":[{"source":"OMIM","code":"254400","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"C84.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B01","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"901411509"},{"source":"ICD-11","code":"XH8R56","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"876349035"},{"source":"MONDO","code":"0009691","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4707404","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"3863","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":[{"symbol":"TNFRSF1B","name":"TNF receptor superfamily member 1B","gene_type":"gene with protein product","association":"Disease-causing somatic mutation(s) in","status":"Assessed","source":"26258847[PMID]","hgnc":"11917","ensembl":"ENSG00000028137","omim":"191191","loci":["1p36.22"]},{"symbol":"CD28","name":"CD28 molecule","gene_type":"gene with protein product","association":"Part of a fusion gene in","status":"Assessed","source":"26258847[PMID]","hgnc":"1653","ensembl":"ENSG00000178562","omim":"186760","loci":["2q33.2"]},{"symbol":"CTLA4","name":"cytotoxic T-lymphocyte associated protein 4","gene_type":"gene with protein product","association":"Part of a fusion gene in","status":"Assessed","source":"26258847[PMID]","hgnc":"2505","ensembl":"ENSG00000163599","omim":"123890","loci":["2q33.2"]}],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial","Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.58,"area":"United States","status":"Validated","source":"32141115[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.5,"area":"Europe","status":"Validated","source":"[EXPERT]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2024-09-20","source":"37124514[PMID]_27871620[PMID]","terms":[{"hpo":"HP:0004332","label":"Abnormal lymphocyte morphology","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000958","label":"Dry skin","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000964","label":"Eczematoid dermatitis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0010783","label":"Erythema","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002665","label":"Lymphoma","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0008069","label":"Neoplasm of the skin","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000989","label":"Pruritus","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0200035","label":"Skin plaque","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000988","label":"Skin rash","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001596","label":"Alopecia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012192","label":"Cutaneous T-cell lymphoma","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025475","label":"Erythematous macule","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001053","label":"Hypopigmented skin patches","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007400","label":"Irregular hyperpigmentation","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002716","label":"Lymphadenopathy","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001029","label":"Poikiloderma","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000492","label":"Abnormal eyelid morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001597","label":"Abnormal nail morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0005561","label":"Abnormality of bone marrow cell morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0007488","label":"Diffuse skin atrophy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000969","label":"Edema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001019","label":"Erythroderma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002240","label":"Hepatomegaly","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000962","label":"Hyperkeratosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0200042","label":"Skin ulcer","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001744","label":"Splenomegaly","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0030166","label":"Night sweats","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Very rare (<4-1%)","criteria":null}]}}