Mycosis fungoides

Summary

Mycosis fungoides (MONDO:0009691) is a disease and 127 clinical trials. Top therapeutic interventions include mogamulizumab, mechlorethamine, and romidepsin. A subtype of primary cutaneous T-cell non-Hodgkin lymphoma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: Unknown (Worldwide) [Orphanet-validated]
• Phenotypes (HPO): 29
• Clinical trials: 127

Clinical features

Epidemiology

Prevalence records

2 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	1-9 / 1 000 000	0.5	Europe	Validated
Annual incidence	1-9 / 1 000 000	0.58	United States	Validated

Signs & symptoms

Clinical features (HPO)

29 HPO clinical features (Orphanet curated; top 29 by frequency):

HPO ID	Term	Frequency
HP:0000958	Dry skin	Very frequent (80-99%)
HP:0000964	Eczematoid dermatitis	Very frequent (80-99%)
HP:0000988	Skin rash	Very frequent (80-99%)
HP:0000989	Pruritus	Very frequent (80-99%)
HP:0002665	Lymphoma	Very frequent (80-99%)
HP:0004332	Abnormal lymphocyte morphology	Very frequent (80-99%)
HP:0008069	Neoplasm of the skin	Very frequent (80-99%)
HP:0010783	Erythema	Very frequent (80-99%)
HP:0200035	Skin plaque	Very frequent (80-99%)
HP:0001029	Poikiloderma	Frequent (30-79%)
HP:0001053	Hypopigmented skin patches	Frequent (30-79%)
HP:0001596	Alopecia	Frequent (30-79%)
HP:0002716	Lymphadenopathy	Frequent (30-79%)
HP:0007400	Irregular hyperpigmentation	Frequent (30-79%)
HP:0012192	Cutaneous T-cell lymphoma	Frequent (30-79%)
HP:0025475	Erythematous macule	Frequent (30-79%)
HP:0000492	Abnormal eyelid morphology	Occasional (5-29%)
HP:0000962	Hyperkeratosis	Occasional (5-29%)
HP:0000969	Edema	Occasional (5-29%)
HP:0001019	Erythroderma	Occasional (5-29%)
HP:0001597	Abnormality of the nail	Occasional (5-29%)
HP:0001744	Splenomegaly	Occasional (5-29%)
HP:0002240	Hepatomegaly	Occasional (5-29%)
HP:0005561	Abnormality of bone marrow cell morphology	Occasional (5-29%)
HP:0007488	Diffuse skin atrophy	Occasional (5-29%)
HP:0200042	Skin ulcer	Occasional (5-29%)
HP:0001824	Weight loss	Very rare (<1-4%)
HP:0001945	Fever	Very rare (<1-4%)
HP:0030166	Night sweats	Very rare (<1-4%)

Identifiers

Disease identifiers

Field	Value
Canonical name	mycosis fungoides
Mondo ID	MONDO:0009691
EFO	EFO:1001051
MeSH	D009182
OMIM	254400
Orphanet	2584
DOID	DOID:8691
ICD-10-CM	C84.0
ICD-11	901411509
NCIT	C3246
SNOMED CT	118618005
UMLS	C0026948
MedGen	7771
GARD	0003863
MedDRA	10028483
NORD	1479
Is cancer (heuristic)	no

Also known as: Alibert-Bazin syndrome · CTCL/ mycosis fungoides · cutaneous T-cell lymphoma/mycosis fungoides · granuloma fungoides · MF · mycosis fungoides · mycosis fungoides (morphologic abnormality) · mycosis fungoides NOS (morphologic abnormality) · mycosis fungoides of unspecified site · mycosis fungoides, Alibert-Bazin type · mycosis fungoides, unspecified site, extranodal and solid organ sites

Data availability: 6 cell lines.

Disease family

This is a subtype of primary cutaneous T-cell non-Hodgkin lymphoma. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › integumentary system disorder › integumentary system cancer › skin cancer › primary cutaneous lymphoma › primary cutaneous T-cell non-Hodgkin lymphoma › mycosis fungoides

Related subtypes (4): primary cutaneous anaplastic large cell lymphoma, Sezary syndrome, mycosis fungoides variant, primary cutaneous gamma-delta t-cell lymphoma

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

Drugs indicated for this disease

6 approved, 6 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.

Drug	Development status
Dexamethasone	Approved (phase 4)
Mechlorethamine	Approved (phase 4)
Methotrexate	Approved (phase 4)
Mogamulizumab	Approved (phase 4)
Prednisolone	Approved (phase 4)
Prednisone	Approved (phase 4)
Bexarotene	Phase 3 (in late-stage trials)
Brentuximab Vedotin	Phase 3 (in late-stage trials)
Hypericin	Phase 3 (in late-stage trials)
Methoxsalen	Phase 3 (in late-stage trials)
Naloxone	Phase 3 (in late-stage trials)
Zanolimumab	Phase 3 (in late-stage trials)

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Alemtuzumab, Atezolizumab, Fludarabine Phosphate, INTERFERON GAMMA-1B, Interferon Alfa, Isotretinoin, Melphalan, Mycophenolate Mofetil, Nelarabine, Pembrolizumab, Ritlecitinib, Rosiglitazone, Tacrolimus Anhydrous, Vorinostat.

Clinical trials & evidence

Clinical trials

Clinical trials: 127.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	39
Not specified	36
PHASE1	24
PHASE1/PHASE2	13
PHASE3	7
PHASE4	4
EARLY_PHASE1	4

Top trials by phase / activity

NCT	Phase	Status	Title
NCT00050999	PHASE4	COMPLETED	Study of ONTAK (Denileukin Diftitox) in Cutaneous T-Cell Lymphoma (CTCL) Patients
NCT00051012	PHASE4	COMPLETED	Study of ONTAK (Denileukin Diftitox) in Previously Treated Cutaneous T-Cell Lymphoma Patients
NCT00221039	PHASE4	COMPLETED	Photopheresis as an Interventional Therapy for the Treatment of CTCL (Cutaneous T-Cell Lymphoma, Mycosis Fungoides) Stage 1A, 1B, 2A
NCT02323659	PHASE4	TERMINATED	Comparison of Methotrexate Versus Interferon-alfa 2b in Patients With Primary Cutaneous T-cell Lymphomas
NCT05205902	PHASE3	NOT_YET_RECRUITING	TOtal Skin Electron Beam Therapy (Low-dose) for Tumor Clone Eradication in Early-stage Mycosis Fungoides
NCT06470451	PHASE3	RECRUITING	Confirmatory Study of Topical HyBryte™ vs. Placebo for the Treatment of CTCL
NCT06479135	PHASE3	RECRUITING	Study of Navtemadlin add-on to Ruxolitinib in JAK Inhibitor-Naïve Patients With Myelofibrosis Who Have a Suboptimal Response to Ruxolitinib
NCT07317700	PHASE3	RECRUITING	A Clinical Trial of Flonoltinib Maleate for Intermediate or High-Risk Myelofibrosis
NCT00127881	PHASE3	TERMINATED	Study of Human Monoclonal Antibody to Treat Mycosis Fungoides and Sezary Syndrome
NCT01578499	PHASE3	COMPLETED	A Phase 3 Trial of Brentuximab Vedotin(SGN-35) Versus Physician’s Choice (Methotrexate or Bexarotene) in Participants With CD30-Positive Cutaneous T-Cell Lymphoma (ALCANZA Study)
NCT02811783	PHASE3	TERMINATED	Naloxone Hydrochloride Study for Relief of Pruritus in Patients With MF or SS Forms of CTCL
NCT01352520	PHASE2	ACTIVE_NOT_RECRUITING	SGN-35 in CD30-positive Lymphoproliferative Disorders (ALCL), Mycosis Fungoides (MF), and Extensive Lymphomatoid Papulosis (LyP)
NCT02978625	PHASE2	ACTIVE_NOT_RECRUITING	Talimogene Laherparepvec and Nivolumab in Treating Patients With Refractory Lymphomas or Advanced or Refractory Non-melanoma Skin Cancers
NCT03011814	PHASE1/PHASE2	ACTIVE_NOT_RECRUITING	Durvalumab With or Without Lenalidomide in Treating Patients With Relapsed or Refractory Cutaneous or Peripheral T Cell Lymphoma
NCT03278782	PHASE1/PHASE2	ACTIVE_NOT_RECRUITING	Study of Pembrolizumab (MK-3475) in Combination With Romidepsin
NCT03385226	PHASE2	ACTIVE_NOT_RECRUITING	A Trial Assessing the Effect of Pembrolizumab Combined with Radiotherapy in Patients with Relapsed, Refractory, Specified Stages of Cutaneous T-cell Lymphoma (CTCL) Mycosis Fungoides (MF)/Sezary Syndrome (SS)
NCT03398161	PHASE2	RECRUITING	Ultra Low Dose Radiation Therapy in Treating Patients With Mycosis Fungoides
NCT03587844	PHASE2	RECRUITING	Dosing of Brentuximab Vedotin for Mycosis Fungoides, Sezary Syndrome Patients
NCT03598998	PHASE1/PHASE2	ACTIVE_NOT_RECRUITING	Pembrolizumab and Pralatrexate in Treating Patients With Relapsed or Refractory Peripheral T-Cell Lymphomas
NCT04256018	PHASE2	RECRUITING	Mogamulizumab + Low-Dose Total Skin Electron Beam Tx in Mycosis Fungoides & Sézary Syndrome
NCT04686682	PHASE1/PHASE2	RECRUITING	A First-in-Human, JAB-8263 in Adult Patients With Advanced Tumors
NCT04960618	PHASE2	ACTIVE_NOT_RECRUITING	Pembrolizumab in Combination With Gemcitabine in People With Advanced Mycosis Fungoides or Sézary Syndrome
NCT05357794	PHASE2	RECRUITING	Effectiveness of Concurrent Ultra-Low-Dose Total-Skin Electron Beam Therapy and Brentuximab Vedotin Given Quarterly Over 12 Months for Patients With Mycosis Fungoides
NCT05680558	PHASE2	RECRUITING	Photopheresis in Early-stage Mycosis Fungoides
NCT06716658	PHASE2	RECRUITING	JAK1 Inhibitor Golidocitnib for the Treatment of Relapsed/Refractory Indolent T/NK-cell Lymphomas
NCT07443306	PHASE2	RECRUITING	A Clinical Trial of Flonoltinib Maleate Tablets in the Treatment of JAK Inhibitor Refractory/Relapsed/Intolerant Patients With Medium to High Risk Myelofibrosis
NCT07529405	PHASE2	NOT_YET_RECRUITING	A Study of VG712 in Patients With Mycosis Fungoides
NCT00038376	PHASE2	COMPLETED	Phase II Study Of Roferon and Accutane For Patients With T-Cell Malignancies
NCT00047060	PHASE1/PHASE2	COMPLETED	Stem Cell Transplant Therapy With Campath-1H for Treating Advanced Mycosis Fungoides and Sezary Syndrome
NCT00091559	PHASE2	COMPLETED	Oral SAHA (Suberoylanilide Hydroxamic Acid) in Advanced Cutaneous T-cell Lymphoma (0683-001)
NCT00129415	PHASE1/PHASE2	TERMINATED	Ultraviolet (UVA and UVB) Light Therapy in the Treatment of Inflammatory Skin Conditions
NCT00157274	PHASE2	UNKNOWN	Study of Alemtuzumab to Treat Advanced Mycosis Fungoides/Sezary Syndrome
NCT00168064	PHASE2	COMPLETED	Safety and Efficacy of Nitrogen Mustard in Treatment of Mycosis Fungoides
NCT00178841	PHASE2	COMPLETED	Combination Drug Study of Bexarotene and Rosiglitazone to Treat CTCL
NCT00185965	PHASE1/PHASE2	COMPLETED	CPG 7909 + Local Radiotherapy in Recurrent Low-Grade Lymphomas
NCT00226993	PHASE1/PHASE2	WITHDRAWN	Phase I/II Study of Intratumoral Injection of CPG 7909, a TLR9 Agonist, Combined With Local Radiation for Patients With Recurrent Mycosis Fungoides.
NCT00535470	PHASE2	COMPLETED	An Open Label Study to Evaluate the Safety and Efficacy of Mechlorethamine(MCH) 0.04% Formulation in Mycosis Fungoides
NCT00611208	PHASE2	COMPLETED	A-dmDT390-bisFv(UCHT1) Immunotoxin Therapy for Patients With Cutaneous T-Cell Lymphoma (CTCL)
NCT01396070	PHASE2	COMPLETED	Brentuximab Vedotin (SGN-35) in Patients With Mycosis Fungoides With Variable CD30 Expression Level
NCT01579318	PHASE2	TERMINATED	Phase II Intratumoral IL12 Plasmid Electroporation in Cutaneous Lymphoma

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
MOGAMULIZUMAB	4	8
MECHLORETHAMINE	4	4
ROMIDEPSIN	4	4
BEXAROTENE	4	3
BRENTUXIMAB VEDOTIN	4	3
RUXOLITINIB	4	3
DENILEUKIN DIFTITOX	4	2
METHOTREXATE	4	2
METHOXSALEN	4	2
PRALATREXATE	4	2
ROSIGLITAZONE	4	2
ALEMTUZUMAB	4	1
AMINOLEVULINIC ACID HYDROCHLORIDE	4	1
BELINOSTAT	4	1
CEFEPIME HYDROCHLORIDE	4	1
CEMIPLIMAB	4	1
COPANLISIB HYDROCHLORIDE	4	1
CYANOCOBALAMIN	4	1
GEMCITABINE	4	1
INTERFERON ALFA-2B	4	1
ISOTRETINOIN	4	1
NALOXONE	4	1
RITLECITINIB	4	1
TALIMOGENE LAHERPAREPVEC	4	1
TRIAMCINOLONE	4	1
HYPERICIN	3	4
AGATOLIMOD SODIUM	3	2
FENRETINIDE	3	1
INTERFERON	3	1
MAGROLIMAB	3	1