ConditionCancer
Cutaneous T-Cell Lymphoma (CTCL)
Cutaneous T-cell lymphoma (CTCL) is a rare type of non-Hodgkin lymphoma in which cancerous T cells, a type of white blood cell, collect in the skin. The most common form, mycosis fungoides, starts as flat, scaly, often itchy patches that look like eczema or psoriasis and progress slowly over years. Sézary syndrome is a rarer, more aggressive form with red skin all over the body and cancer cells in the blood. Early-stage CTCL is usually managed with skin-directed treatments and has a near-normal life expectancy.
Also called: CTCL, mycosis fungoides, Sézary syndrome, cutaneous lymphoma
At a glance
- What it is
- A lymphoma of T cells that mainly affects the skin.
- Main types
- Mycosis fungoides (most common) and Sézary syndrome. Other rarer subtypes exist.
- Early signs
- Persistent flat, scaly, pink or red patches, often on the buttocks, hips and trunk, that do not respond well to usual eczema treatment.
- Diagnosis
- Skin biopsies, often repeated, plus blood tests and sometimes scans.
- Treatment
- Skin creams, light therapy, radiation, and systemic therapies for advanced disease.
When to get help
Call 911 or go to the ER
- Fever, chills and feeling very unwell with widespread red, broken or weeping skin (possible serious skin infection or sepsis)
See a doctor within a day or two
- Red, scaly skin covering most of the body, often with intense itching, peeling and feeling cold (possible Sézary syndrome or erythroderma)
- Rapidly growing lumps (tumors) in the skin or ulcers that will not heal
- New swollen lymph nodes, weight loss or night sweats with a skin rash
Book a routine appointment
- A scaly rash or patches that persist for months despite eczema or psoriasis treatment
- Patches in sun-protected areas, such as the buttocks, hips, upper thighs or breasts
- Thinned, wrinkled or discolored patches of skin that come and go
- Known CTCL with new skin changes, itching or lumps
What CTCL is
T cells normally travel to the skin to fight infection. In CTCL, a group of abnormal T cells multiplies and settles in the skin. Unlike most lymphomas, which start in lymph nodes, CTCL starts and often stays in the skin for years. In advanced stages it can spread to lymph nodes, blood and internal organs.
Types
| Type | Features |
|---|---|
| Mycosis fungoides (MF) | About half or more of CTCL. Patches, then plaques, then sometimes tumors over years. Usually slow |
| Sézary syndrome | Widespread red skin (erythroderma), enlarged lymph nodes and lymphoma cells in the blood. Intense itching. More aggressive |
| Primary cutaneous CD30-positive disorders | Including lymphomatoid papulosis and anaplastic large cell lymphoma, often with crops of bumps or nodules |
| Other rare subtypes | Folliculotropic MF, subcutaneous panniculitis-like T-cell lymphoma and others |
Symptoms
Mycosis fungoides
- Patches: flat, pink, red or brown areas with fine scale, often on the buttocks, hips, upper thighs, lower trunk and breasts. On darker skin they may look lighter or darker than surrounding skin.
- Plaques: raised, thicker, scaly areas.
- Tumors: dome-shaped lumps that may ulcerate.
- Itching, which can be severe
- Hair loss in affected areas (especially folliculotropic MF)
Sézary syndrome
- Red, scaly skin over most of the body (erythroderma)
- Severe itching
- Thickened skin on palms and soles, nail changes, hair loss
- Swollen lymph nodes
- Feeling cold from heat loss through the skin
Because early MF looks like eczema, psoriasis or fungal infections, diagnosis is often delayed for years.
Causes and risk factors
The cause is unknown. CTCL is not contagious and is not caused by eczema or psoriasis. It is more common in people over 50, in men, and in Black Americans, who also tend to be diagnosed at a younger age.
Diagnosis
- Skin biopsies: small samples examined under the microscope, with special stains for T-cell markers. Several biopsies over time are often needed because early changes are subtle.
- T-cell receptor gene rearrangement testing shows whether T cells are clonal (all from one abnormal cell).
- Blood tests: blood count, flow cytometry to look for Sézary cells, and LDH.
- Lymph node biopsy if lymph nodes are swollen.
- Imaging: CT or PET-CT in more advanced stages.
Stages
Mycosis fungoides and Sézary syndrome are staged using the TNMB system (skin, nodes, organs, blood):
| Stage | Description |
|---|---|
| IA | Patches or plaques on less than 10 percent of the skin |
| IB | Patches or plaques on 10 percent or more of the skin |
| IIA | Skin patches or plaques with enlarged but not cancerous lymph nodes |
| IIB | One or more skin tumors |
| III | Erythroderma covering most of the body |
| IV | Significant blood involvement (Sézary syndrome), lymph node or organ spread |
Treatment
CTCL is usually treated as a chronic condition. The aim is to control skin disease, relieve itching and maintain quality of life.
Skin-directed therapies (early stages)
- Topical corticosteroids
- Topical chemotherapy (mechlorethamine gel) and topical retinoids (bexarotene)
- Phototherapy: narrowband UVB for thin patches, PUVA for thicker plaques
- Localized radiation therapy for individual plaques or tumors
- Total skin electron beam therapy for widespread skin disease
Systemic therapies (advanced or resistant disease)
- Oral retinoids (bexarotene) and interferon
- Extracorporeal photopheresis, which treats blood cells with light outside the body, especially for Sézary syndrome
- Targeted antibodies such as mogamulizumab and brentuximab vedotin (for CD30-positive disease)
- Histone deacetylase inhibitors, low-dose methotrexate and chemotherapy
- Allogeneic stem cell transplant for selected people with advanced disease
Supportive care
Itch control with moisturizers, antihistamines and other medicines, skin care to prevent infection, and psychological support.
Outlook
Early-stage mycosis fungoides (IA) usually progresses slowly, and many people have a normal or near-normal life expectancy. Survival falls with more advanced stages, and Sézary syndrome has a poorer outlook, although newer treatments have improved control. Regular follow-up with a dermatologist or cutaneous lymphoma specialist is important.
Frequently asked questions
What are the first signs of cutaneous T-cell lymphoma?
The first signs are usually flat, scaly, pink or red patches that may itch, often on the buttocks, hips, upper thighs or trunk. They can look like eczema or psoriasis but persist despite usual treatment. A skin biopsy is needed to diagnose CTCL.
Is cutaneous T-cell lymphoma a type of skin cancer?
It is a cancer that shows up in the skin, but it is not a skin cancer in the usual sense. It is a type of non-Hodgkin lymphoma, a cancer of white blood cells called T cells, which collect in the skin rather than starting in the lymph nodes.
What is the difference between mycosis fungoides and Sézary syndrome?
Mycosis fungoides is the most common form of CTCL and usually progresses slowly from skin patches to plaques and sometimes tumors. Sézary syndrome is rarer and more aggressive, with red skin covering most of the body, enlarged lymph nodes and lymphoma cells in the blood.
Is cutaneous T-cell lymphoma curable?
Early-stage disease can sometimes go into long-term remission, and many people live normal lifespans with skin-directed treatment. Advanced CTCL is usually not curable with standard therapy, but it can often be controlled for years. Stem cell transplant offers a chance of cure for selected people.
Sources
This guide was written from current patient and clinical guidance, including:
- National Cancer Institute: Mycosis Fungoides and Other Cutaneous T-Cell Lymphomas Treatment (PDQ)
- NCCN Guidelines for Patients: Primary Cutaneous Lymphomas
- Leukemia & Lymphoma Society: Cutaneous T-cell lymphoma
- American Academy of Dermatology: Cutaneous T-cell lymphoma
- Cutaneous Lymphoma Foundation: Understanding CTCL