{"entity":{"id":"mycosis-fungoides","kind":"cancer","name":"Mycosis fungoides","aka":["MF","Mycosis fungoides / Sezary syndrome (CTCL)","Alibert-Bazin syndrome","Granuloma fungoides","Folliculotropic mycosis fungoides","Pagetoid reticulosis","Granulomatous slack skin"],"tldr":"The commonest cutaneous T-cell lymphoma, and a disease that behaves like a long-term skin condition for most of the people who have it: flat scaly patches that have often been treated as eczema or psoriasis for years before anyone takes a biopsy. In its early stages life expectancy is close to normal, and the treatment is creams and light rather than chemotherapy.","summary":"What it is. A lymphoma of mature T cells that lives in the skin. It begins as flat, scaly, often itchy patches, classically on parts of the body the sun does not reach, and over years some people develop raised plaques and then tumours. A minority develop redness over most of the body, and a minority have lymphoma cells in the blood, at which point it overlaps with Sezary syndrome. It is the commonest of the cutaneous T-cell lymphomas.\n\nThe diagnosis is slow and that is normal. Early mycosis fungoides looks like eczema, psoriasis or a drug rash, and the biopsy is often not diagnostic until the disease is established. Several biopsies over several years, read by a dermatopathologist alongside photographs and the history, are the usual route to the diagnosis, and that is not a failure of care. WHO-HAEM5 states the principle for the whole group of skin lymphomas: because the appearances overlap, dermatological examination and clinical photographic documentation are indispensable.\n\nHow it is staged, and why the stage matters more than usual. The system in use is the revised ISCL and EORTC staging of 2007, which classifies the skin (patches or plaques covering under or over a tenth of the body surface, tumours, or redness over most of the body), the lymph nodes, the internal organs and the blood. The validation study in 1,502 patients confirmed that the resulting stages separate survival, and found something the earlier system had missed: among people with early skin disease, those with patches alone did significantly better than those with patches and plaques, which is why the stage now records the difference.\n\nThe things that predict the course. In that study, advanced skin stage, the presence of the tumour clone in the blood without full-blown Sezary cells, a raised lactate dehydrogenase and the folliculotropic form were each independently associated with worse survival. In the other direction, the pale form, the mottled form and mycosis fungoides occurring with lymphomatoid papulosis were associated with better survival and less risk of progression. Large-cell transformation, where the cells in a lesion become large and more aggressive, is a specific event that changes the treatment.\n\nThe variants. WHO-HAEM5 keeps the variants of mycosis fungoides as subtypes and makes one change: within the folliculotropic form, which involves the hair follicles and is harder to treat because creams and light do not reach deeply enough, early and advanced clinical patterns are now distinguished because they behave differently.\n\nWhat it means for a person. Early-stage mycosis fungoides, which is most of it, has a life expectancy close to that of the general population and is treated with creams, light and occasionally small doses of radiotherapy, over decades. Treating early disease with chemotherapy shortens neither the disease nor anything else and causes harm. Advanced disease, meaning tumours, widespread redness, or involvement of the nodes, organs or blood, needs systemic treatment and is a different situation: in a cohort of 168 people with advanced mycosis fungoides or Sezary syndrome, median survival was 2.47 years, and those started on biological treatments rather than combination chemotherapy lived longer. The treatment rows for every stage are on the cutaneous T-cell lymphoma page, which is the hub this record sits under.","asOf":"2026-09-29","wikipedia":"https://en.wikipedia.org/wiki/Mycosis_fungoides","links":[{"label":"WHO Classification of Haematolymphoid Tumours, 5th edition: lymphoid neoplasms (Alaggio, Leukemia 2022)","url":"https://doi.org/10.1038/s41375-022-01620-2"},{"label":"International Consensus Classification of Mature Lymphoid Neoplasms (Campo, Blood 2022)","url":"https://doi.org/10.1182/blood.2022015851"},{"label":"Survival outcomes and prognostic factors in mycosis fungoides and Sezary syndrome, validation of the revised ISCL/EORTC staging proposal in 1,502 patients (Agar, J Clin Oncol 2010)","url":"https://doi.org/10.1200/JCO.2009.27.7665"},{"label":"Advanced-stage mycosis fungoides and Sezary syndrome, survival and response to treatment in 168 patients (Clinical Lymphoma Myeloma and Leukemia 2015)","url":"https://doi.org/10.1016/j.clml.2015.02.027"},{"label":"Lymphoma incidence, survival and prevalence 2004 to 2014, subtype analyses from the UK Haematological Malignancy Research Network (Smith, Br J Cancer 2015)","url":"https://doi.org/10.1038/bjc.2015.94"},{"label":"NCI PDQ: adult non-Hodgkin lymphoma treatment (health professional version)","url":"https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq"}],"tags":["heme","lymphoma","subtype-page"],"related":["cutaneous-t-cell-lymphoma","sezary-syndrome","primary-cutaneous-anaplastic-large-cell-lymphoma","lymphomatoid-papulosis","peripheral-t-cell-lymphoma"],"cancers":[],"sections":[],"technologies":["histopathology-ihc","total-skin-electron-therapy","palliative-radiotherapy","allogeneic-hsct"],"targets":["cd30"],"drugs":["mechlorethamine","bexarotene","mogamulizumab","brentuximab-vedotin","methotrexate","interferon-alfa","romidepsin","vorinostat","denileukin-diftitox","methoxsalen-ecp","imiquimod","pegylated-liposomal-doxorubicin"],"companies":[],"institutions":[],"pathways":[],"terms":["lymphoma-tx-skin-directed-therapy","lymphoma-indolent-versus-aggressive","lymphoma-classification-2022","lymphoma-nodal-versus-extranodal","lymphoma-tx-radiotherapy","lymphoma-transformation","lymphoma-tx-uk-access"],"trials":["alcanza","mavoric"],"people":[],"bottlenecks":[],"keyPapers":["paper-olsen-mycosis-fungoides-staging-blood-2007"],"journals":[],"dependsOn":[],"notes":[],"group":"haematologic","burden":"In the United Kingdom population series that reports lymphoma by subtype, 39 of 5,796 lymphomas were mycosis fungoides, a European age-standardised rate of 0.12 per 100,000 a year, with men affected about 2.7 times as often as women and a median age at diagnosis of 65.8 years; five-year relative survival in that series was 86.6 per cent. In the international cohort of 1,502 people with mycosis fungoides or Sezary syndrome that validated the current staging system, the mean age at diagnosis was 54 years, 71 per cent presented with early-stage disease, the disease progressed in 34 per cent and 26 per cent died of it.","subtypes":["Classic mycosis fungoides, with patches, plaques and sometimes tumours","Folliculotropic mycosis fungoides, which involves the hair follicles; WHO-HAEM5 distinguishes early from advanced clinical patterns within it","Pagetoid reticulosis, a localised form","Granulomatous slack skin","Hypopigmented, poikilodermatous and other clinical variants, several of which carry a better outlook","Large-cell transformation, which is an event rather than a variant and changes the treatment"],"biomarkers":["The revised ISCL and EORTC stage of 2007, covering skin, nodes, viscera and blood","Patches alone against patches and plaques within early skin stage, which separates survival","The folliculotropic variant, independently associated with worse survival","The tumour clone detectable in the blood without Sezary cells, which is independently associated with worse survival","Lactate dehydrogenase","Large-cell transformation on a repeat biopsy of a changing lesion","CD30 expression, which decides whether brentuximab vedotin is an option"],"standardOfCare":[{"setting":"Mycosis fungoides: staging first, because stage decides everything","approach":"Mycosis fungoides is staged by the ISCL/EORTC TNMB system, which classifies skin (patches or plaques covering under or over 10 per cent of the body surface, tumours, erythroderma), nodes, viscera and blood. The staging revision of 2007 remains the basis of treatment choice and trial eligibility, and a validation study in a large cohort confirmed that the resulting stages separate survival.\n\nWhy it matters more here than in most cancers: early-stage mycosis fungoides (stage IA to IIA, patches and plaques without tumours or blood involvement) has a life expectancy close to that of the general population and is treated with creams and light, while advanced disease (tumour stage, erythroderma, nodal or blood involvement) needs systemic treatment. Treating early disease with chemotherapy shortens neither the disease nor anything else and causes harm, which is the single most important thing to get right.\n\nThe diagnosis itself is often slow, because early mycosis fungoides looks like eczema or psoriasis for years and the biopsy is frequently non-diagnostic until the disease is established. Repeated biopsies over time, read by a dermatopathologist, are normal and are not a failure.","refs":["paper-olsen-mycosis-fungoides-staging-blood-2007","lymphoma-tx-skin-directed-therapy","lymphoma-type"],"guideline":{"version":"NCCN Primary Cutaneous Lymphomas; ESMO; ISCL/EORTC staging","url":"https://doi.org/10.1200/JCO.2009.27.7665"}},{"setting":"Early-stage mycosis fungoides (IA to IIA): skin-directed treatment only","approach":"The aim is control of the skin with the least treatment that achieves it, over decades.\n\nTopical corticosteroids, potent or very potent, are first line for patches and plaques and clear a large proportion. Topical mechlorethamine (chlormethine) gel, approved in the United States on 23 August 2013 for stage IA and IB mycosis fungoides-type cutaneous T-cell lymphoma after prior skin-directed therapy, cleared index lesions in its pivotal randomised trial in 58.5 per cent of patients against 47.7 per cent with the traditional compounded ointment, meeting the non-inferiority bar; contact dermatitis is the main problem and is managed by reducing frequency rather than stopping. Topical bexarotene gel and topical carmustine are alternatives. Imiquimod is used for a small number of resistant plaques.\n\nPhototherapy is the mainstay for widespread patch and plaque disease: narrowband UVB for patches and thin plaques, PUVA (psoralen with UVA) for thicker plaques and follicular disease, given two or three times a week at a dermatology unit and then tapered to a maintenance schedule. Cumulative UV dose carries its own long-term skin cancer risk, which is the reason for tapering.\n\nLocalised radiotherapy at low dose, often 8 Gy in two fractions, clears an individual tumour or a resistant plaque quickly and can be repeated. Total skin electron beam therapy treats the whole skin surface and is reserved for extensive disease; low-dose schedules of 10 to 12 Gy are now preferred to the historic 30 to 36 Gy because they can be repeated.","refs":["mechlorethamine","bexarotene","carmustine","imiquimod","total-skin-electron-therapy","palliative-radiotherapy","lymphoma-tx-skin-directed-therapy","lymphoma-tx-radiotherapy","paper-study-201-jama-dermatol-2013"],"guideline":{"version":"NCCN Primary Cutaneous Lymphomas; ESMO; BAD/BSH primary cutaneous lymphoma guideline","url":"https://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq"}},{"setting":"Advanced mycosis fungoides (IIB to IVB): systemic treatment, and what to reach for first","approach":"Systemic treatment is indicated for tumour-stage disease, erythroderma, nodal or visceral involvement, blood involvement, or skin disease that has failed skin-directed therapy. Single agents are preferred to combination chemotherapy, which produces fast responses that do not last and damages the immune system in a patient already prone to skin infection and sepsis.\n\nRetinoids and interferon. Oral bexarotene and interferon alfa are long-standing options, often combined with phototherapy; both are slow-acting and require monitoring of lipids and thyroid function in the case of bexarotene.\n\nMethotrexate at low weekly oral dose, which is inexpensive, familiar and effective in a proportion.\n\nMogamulizumab, an anti-CCR4 antibody approved in the United States on 8 August 2018. MAVORIC randomised 372 patients with previously treated mycosis fungoides or Sezary syndrome to mogamulizumab or vorinostat: median progression-free survival 7.7 against 3.1 months (hazard ratio 0.53) and overall response 28 against 5 per cent, with a response in the blood compartment of 68 per cent. It is therefore the drug of choice where the blood is involved.\n\nBrentuximab vedotin for CD30-expressing disease. ALCANZA randomised patients with CD30-positive mycosis fungoides or primary cutaneous anaplastic large cell lymphoma to brentuximab vedotin or to physician's choice of methotrexate or bexarotene: objective response lasting four months or more was 56.3 against 12.5 per cent and median progression-free survival 16.7 against 3.5 months. Peripheral neuropathy is the limiting toxicity.\n\nOther options: romidepsin, denileukin diftitox, which returned in 2024 as Lymphir for relapsed or refractory stage I to III disease after at least one systemic therapy, gemcitabine, pegylated liposomal doxorubicin, and allogeneic transplant with reduced-intensity conditioning, which is the only treatment that produces long remissions in advanced disease and is offered to younger fit patients.","refs":["paper-mavoric-mogamulizumab-lancet-oncol-2018","paper-alcanza-brentuximab-vedotin-lancet-2017","mogamulizumab","brentuximab-vedotin","bexarotene","interferon-alfa","methotrexate","romidepsin","vorinostat","denileukin-diftitox","gemcitabine","pegylated-liposomal-doxorubicin","allogeneic-hsct","lymphoma-tx-skin-directed-therapy"],"guideline":{"version":"NCCN Primary Cutaneous Lymphomas; ESMO; MAVORIC, ALCANZA","url":"https://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq"}},{"setting":"Living with mycosis fungoides: itch, infection and the things that make the difference day to day","approach":"Itch is the symptom patients rank as the worst and it is undertreated. Emollients applied several times a day, potent topical steroids, sedating antihistamines at night, gabapentin or pregabalin for neuropathic itch, and aprepitant or mirtazapine in resistant cases. Controlling the disease is the most effective anti-itch treatment, which is an argument for treating skin disease that is not otherwise dangerous.\n\nInfection. Staphylococcus aureus colonises broken skin and is a common cause of both flares and sepsis; swabs, topical antiseptics, bleach baths and prompt antibiotics for cellulitis are part of routine care, and a patient with advanced disease and a fever is treated as an emergency.\n\nSkin care. Soap substitutes, lukewarm baths, cotton clothing, and avoidance of overheating. Photoprotection after phototherapy or radiotherapy.\n\nThe long view. Early-stage mycosis fungoides is a chronic skin disease to be managed rather than an emergency, and most people with it die of something else. That is worth saying out loud at diagnosis, because the word lymphoma does not sound like that.","refs":["lymphoma-tx-skin-directed-therapy","palliative-care","mechlorethamine","bexarotene"],"guideline":{"version":"NCCN Primary Cutaneous Lymphomas; BAD/BSH primary cutaneous lymphoma guideline","url":"https://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq"}}],"stateOfArt":[],"history":[{"year":1806,"title":"Described by Alibert","note":"Jean-Louis Alibert described the mushroom-like tumours that gave the disease its misleading name; it has nothing to do with fungal infection.","refs":[]},{"year":2007,"title":"The ISCL and EORTC staging revision","note":"The staging system was revised to classify skin, node, visceral and blood involvement separately, which remains the basis of treatment choice and trial eligibility.","refs":["paper-olsen-mycosis-fungoides-staging-blood-2007"]},{"year":2010,"title":"The staging validated in 1,502 patients","note":"The revised stages were shown to separate survival, and patients with patches alone were shown to do significantly better than those with patches and plaques within the same early stage.","refs":[]},{"year":2022,"title":"The folliculotropic form split by clinical pattern","note":"WHO-HAEM5 kept the variants of mycosis fungoides as subtypes and distinguished early from advanced clinical patterns within the folliculotropic category, because their outcomes differ.","refs":["lymphoma-classification-2022"]}],"pipeline":[],"openProblems":["Nothing has been shown to change the natural history of early-stage mycosis fungoides, so every treatment given in early disease is for symptoms, and over-treatment is the commonest harm.","The time from first rash to diagnosis is measured in years, and no test identifies early mycosis fungoides reliably on a single biopsy.","Itch is the symptom people with this disease rank as the worst and there is no randomised trial of any treatment for it in mycosis fungoides.","Allogeneic transplant is the only treatment that produces long remissions in advanced disease, and no trial defines who should have it or when.","Early-stage mycosis fungoides has a life expectancy close to that of the general population and no treatment has been shown to change it, so every intervention in early disease is for symptoms; over-treatment is the commonest harm in this disease.","Itch is the symptom patients rank worst and there is no randomised trial of any anti-itch treatment in mycosis fungoides.","Allogeneic transplant is the only treatment that produces long remissions in advanced cutaneous T-cell lymphoma, and there is no trial defining who should have it or when."],"parent":"cutaneous-t-cell-lymphoma"},"route":"/cancers/mycosis-fungoides/","neighbours":{"cancer":[{"id":"cutaneous-t-cell-lymphoma","kind":"cancer","name":"Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome)","route":"/cancers/cutaneous-t-cell-lymphoma/"},{"id":"lymphomatoid-papulosis","kind":"cancer","name":"Lymphomatoid papulosis","route":"/cancers/lymphomatoid-papulosis/"},{"id":"non-hodgkin-lymphoma","kind":"cancer","name":"Non-Hodgkin lymphoma (all types)","route":"/cancers/non-hodgkin-lymphoma/"},{"id":"peripheral-t-cell-lymphoma","kind":"cancer","name":"Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma)","route":"/cancers/peripheral-t-cell-lymphoma/"},{"id":"primary-cutaneous-anaplastic-large-cell-lymphoma","kind":"cancer","name":"Primary cutaneous anaplastic large cell lymphoma","route":"/cancers/primary-cutaneous-anaplastic-large-cell-lymphoma/"},{"id":"sezary-syndrome","kind":"cancer","name":"Sezary syndrome","route":"/cancers/sezary-syndrome/"}],"technology":[{"id":"allogeneic-hsct","kind":"technology","name":"Allogeneic stem cell transplantation","route":"/technologies/allogeneic-hsct/"},{"id":"palliative-care","kind":"technology","name":"Early integrated palliative care","route":"/technologies/palliative-care/"},{"id":"histopathology-ihc","kind":"technology","name":"Histopathology & immunohistochemistry","route":"/technologies/histopathology-ihc/"},{"id":"palliative-radiotherapy","kind":"technology","name":"Palliative radiotherapy","route":"/technologies/palliative-radiotherapy/"},{"id":"total-skin-electron-therapy","kind":"technology","name":"Total skin electron beam therapy","route":"/technologies/total-skin-electron-therapy/"}],"target":[{"id":"cd30","kind":"target","name":"CD30","route":"/targets/cd30/"}],"drug":[{"id":"bexarotene","kind":"drug","name":"Bexarotene","route":"/drugs/bexarotene/"},{"id":"brentuximab-vedotin","kind":"drug","name":"Brentuximab vedotin","route":"/drugs/brentuximab-vedotin/"},{"id":"carmustine","kind":"drug","name":"Carmustine","route":"/drugs/carmustine/"},{"id":"denileukin-diftitox","kind":"drug","name":"Denileukin diftitox","route":"/drugs/denileukin-diftitox/"},{"id":"gemcitabine","kind":"drug","name":"Gemcitabine","route":"/drugs/gemcitabine/"},{"id":"imiquimod","kind":"drug","name":"Imiquimod","route":"/drugs/imiquimod/"},{"id":"interferon-alfa","kind":"drug","name":"Interferon alfa-2a/2b","route":"/drugs/interferon-alfa/"},{"id":"mechlorethamine","kind":"drug","name":"Mechlorethamine (chlormethine)","route":"/drugs/mechlorethamine/"},{"id":"methotrexate","kind":"drug","name":"Methotrexate","route":"/drugs/methotrexate/"},{"id":"methoxsalen-ecp","kind":"drug","name":"Methoxsalen (extracorporeal photopheresis)","route":"/drugs/methoxsalen-ecp/"},{"id":"mogamulizumab","kind":"drug","name":"Mogamulizumab","route":"/drugs/mogamulizumab/"},{"id":"pegylated-liposomal-doxorubicin","kind":"drug","name":"Pegylated liposomal doxorubicin","route":"/drugs/pegylated-liposomal-doxorubicin/"},{"id":"romidepsin","kind":"drug","name":"Romidepsin","route":"/drugs/romidepsin/"},{"id":"vorinostat","kind":"drug","name":"Vorinostat","route":"/drugs/vorinostat/"}],"term":[{"id":"lymphoma-indolent-versus-aggressive","kind":"term","name":"Indolent and aggressive lymphoma","route":"/terms/lymphoma-indolent-versus-aggressive/"},{"id":"lymphoma-type","kind":"term","name":"Lymphoma (tissue type)","route":"/terms/lymphoma-type/"},{"id":"lymphoma-nodal-versus-extranodal","kind":"term","name":"Nodal and extranodal lymphoma","route":"/terms/lymphoma-nodal-versus-extranodal/"},{"id":"lymphoma-tx-radiotherapy","kind":"term","name":"Radiotherapy in lymphoma: involved-site fields, 24 Gy, 4 Gy and total skin electron therapy","route":"/terms/lymphoma-tx-radiotherapy/"},{"id":"lymphoma-tx-skin-directed-therapy","kind":"term","name":"Skin-directed therapy in mycosis fungoides: creams, light and small radiotherapy fields","route":"/terms/lymphoma-tx-skin-directed-therapy/"},{"id":"lymphoma-classification-2022","kind":"term","name":"The two lymphoma classifications of 2022 (WHO-HAEM5 and ICC)","route":"/terms/lymphoma-classification-2022/"},{"id":"lymphoma-transformation","kind":"term","name":"Transformation of an indolent lymphoma","route":"/terms/lymphoma-transformation/"},{"id":"lymphoma-tx-uk-access","kind":"term","name":"What the NHS in England funds for lymphoma, appraisal by appraisal","route":"/terms/lymphoma-tx-uk-access/"}],"trial":[{"id":"alcanza","kind":"trial","name":"ALCANZA","route":"/trials/alcanza/"},{"id":"mavoric","kind":"trial","name":"MAVORIC","route":"/trials/mavoric/"}],"paper":[{"id":"paper-alcanza-brentuximab-vedotin-lancet-2017","kind":"paper","name":"ALCANZA: brentuximab vedotin versus physician's choice in CD30-positive cutaneous T-cell lymphoma","route":"/key-papers/paper-alcanza-brentuximab-vedotin-lancet-2017/"},{"id":"paper-mavoric-mogamulizumab-lancet-oncol-2018","kind":"paper","name":"MAVORIC: mogamulizumab versus vorinostat in previously treated cutaneous T-cell lymphoma","route":"/key-papers/paper-mavoric-mogamulizumab-lancet-oncol-2018/"},{"id":"paper-olsen-mycosis-fungoides-staging-blood-2007","kind":"paper","name":"Revisions to the staging and classification of mycosis fungoides and Sezary syndrome (ISCL/EORTC)","route":"/key-papers/paper-olsen-mycosis-fungoides-staging-blood-2007/"},{"id":"paper-study-201-jama-dermatol-2013","kind":"paper","name":"Topical chemotherapy in cutaneous T-cell lymphoma: positive results of a randomized, controlled, multicenter trial testing the efficacy and safety of a novel mechlorethamine, 0.02%, gel in mycosis fungoides","route":"/key-papers/paper-study-201-jama-dermatol-2013/"}]}}