Primary cutaneous (skin) lymphoma
Primary cutaneous lymphoma is non-Hodgkin lymphoma that affects the skin.
What is primary cutaneous lymphoma?
Primary cutaneous lymphoma is also called skin lymphoma. It is non-Hodgkin lymphoma that starts in the skin and mainly affects the skin.
Skin lymphoma develops when blood cells called lymphocytes become abnormal. These cells normally help fight infection. The abnormal cells build up in the skin and may cause symptoms.
Skin lymphoma is rare. There are different types. The types are split into 2 groups:
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Cutaneous T-cell skin lymphomas
About 7 in 10 skin lymphomas (70%) develop from a type of lymphocyte called a T-cell. The most common T-cell skin lymphomas are mycosis fungoides and Sézary syndrome.
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Cutaneous B-cell skin lymphomas
B-cell skin lymphoma is less common. About 3 in 10 skin lymphomas (30%) develop from a type of lymphocyte called a B-cell. The most common types are follicle centre lymphoma and marginal zone lymphoma. These are slow growing (low grade) lymphomas.
Mycosis fungoides
Mycosis fungoides (MF) is the most common type of skin lymphoma.
It is usually very slow growing. It usually only affects the skin. This means that for most people, MF stays under control for many years. In a small number of people, MF may spread to other parts of the body over time.
MF is often difficult to diagnose. There are several different subtypes.
Sézary syndrome
Sézary syndrome (SS) affects the skin, but the abnormal lymphocytes circulate in the blood. This means SS may be found in other areas of the body, such as lymph nodes. SS is usually faster growing. It usually causes more severe symptoms than MF.
Low grade B-cell skin lymphomas
Follicle centre lymphoma and marginal zone lymphoma are both slow growing (low grade). When they start in the skin, they usually only affect the skin. Some people will not need treatment. Some people may have treatment to cure the lymphoma or manage symptoms.
Primary cutaneous diffuse large B-cell lymphoma
Diffuse large B-cell lymphoma (DLBCL) that starts in the skin is rare. It usually grows quickly and may need treatment straight away. It is usually treated in a similar way to DLBCL that has started in other parts of the body. The information on this page may not be relevant. But we have separate information about DLBCL and how it is treated.
Related pages
Skin lymphoma symptoms
In the early stages, skin lymphoma often looks like other, more common skin conditions such as eczema or psoriasis. This may mean it takes time to diagnose.
Skin lymphoma symptoms may include:
- flat red, rash-like patches
- itchy, sometimes painful areas
- raised patches (plaques)
- lumps under the skin
- a painless swelling where the abnormal cells have built up in a lymph node.
What does skin lymphoma look like?
It is not always possible to tell whether skin changes are cancer just by looking at the skin. There are many other skin conditions that are not lymphoma.
Ask your GP if you are worried about skin changes or have ongoing, new or unexplained skin symptoms. We are also here if you need someone to talk to. You can:
- call the Macmillan Support Line free on 0808 808 00 00
- chat to our specialists online.
If you are searching for photos of skin lymphoma, Lymphoma Action has more information.
Related pages
Skin lymphoma causes
Doctors do not know what causes skin lymphoma. Like other cancers, it is not infectious. You cannot spread it to other people.
Mycosis fungoides and Sézary syndrome more commonly affect people aged 60 and over. These types are more common in men than women.
Low grade B-cell skin lymphomas affect all age groups. Some cases of marginal zone lymphoma are linked to the infection that causes Lyme disease.
We have more general information about risk factors of non-Hodgkin lymphoma.
Skin lymphoma diagnosis
If your GP thinks your symptoms could be cancer, they can refer you to a specialist at hospital. For skin symptoms, this is usually a doctor who specialises in treating skin diseases. They are called a dermatologist.
The most common test for diagnosing skin lymphoma is a biopsy. This is when a sample of tissue is taken and then tested to find out more. You usually have a biopsy in an outpatient clinic. Your doctor will give you more information. They can tell you when to expect your results and how you will get them.
Skin lymphoma can be difficult to diagnose. You may need another biopsy or more tests before doctors can give a diagnosis.
If the biopsy shows lymphoma, you may have a blood test. This is to check for signs of lymphoma cells in the blood. You may also have other tests and scans. You can read more about further tests in our information about non-Hodgkin lymphoma.
Getting a skin lymphoma diagnosis can take time, and waiting for test results can be stressful. During this time you may find it helpful to talk to family members or a close friend. Your specialist nurse can also provide support. Or you can talk to one of our cancer support specialists free on 0808 808 00 00, 7 days a week, 8am to 8pm.
Related pages
Staging and grading of skin lymphoma
Your test results give your cancer team more information about the lymphoma. This includes information about the type, stage and grade. This information helps your team plan the right treatment for you.
Staging
The stage of skin lymphoma describes how much of the skin is affected and whether the lymphoma has spread anywhere else.
Different staging systems are used depending on the type of lymphoma. This section gives a simple overview. Talk to your cancer team for more detailed information about your diagnosis.
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Number staging system for MF or SS
If you are diagnosed with mycosis fungoides (MF) or Sézary syndrome (SS), the stage may be described using a number system.
- Stage 1 – describes early MF or SS. This means the lymphoma is only affecting the skin. There are patches of skin changes.
- Stage 2 – describes when some lymph nodes are swollen or contain lymphoma, or skin tumours have developed.
- Stage 3 – describes when more than 80% of the skin is inflamed. This is called erythroderma.
- Stage 4 – describes advanced MF or SS. This means the lymphoma has spread to other organs in the body, such as the liver, lungs or bone marrow.
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TNM staging system for MF or SS
MF or SS can also be described using the TNM staging system. This system is used to describe many cancer types. This is how it is used for MF and SS:
- Tumour (T) – this describes how much of the skin is affected. T1 means skin changes affect less than 10% of the skin. T4 means more than 80% of the skin is inflamed (erythroderma).
- Nodes (N) – N0 means there are no swollen lymph nodes. N1, N2 and N3 mean the lymph nodes are swollen or contain lymphoma.
- Metastases (M) – M1 means the lymphoma has spread to another part of the body, such as the liver, lungs or bone marrow. This is called metastatic cancer. M0 means no sign of this.
- Blood (B) – this measure is used for SS. It describes the number of lymphoma cells in the blood. B0 means no lymphoma cells or a very small amount. B1 and B2 mean larger numbers.
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TNM staging system for other skin lymphomas
This is how TNM is used for skin lymphomas that are not MF or SS:
- Tumour – this describes how much of the skin is affected. T1 means one patch of skin. T2 means several patches in the same area of the body. T3 means patches in several different areas of the body.
- Nodes – N0 means there are no affected lymph nodes. N1, N2 and N3 mean lymph nodes are affected by lymphoma.
- Metastases – M1 means the lymphoma has spread to another part of the body (metastatic cancer). M0 means no sign of this.
Grading
Grading describes how quickly the lymphoma cells are likely to grow. Low grade lymphoma grows very slowly. High grade lymphoma grows more quickly.
Treatment for skin lymphoma
A team of specialists will meet to discuss the best possible treatment for you. They are called a multidisciplinary team (MDT).
After the MDT meeting, you will usually meet with your cancer doctor and specialist nurse. They will explain the different treatments and their side effects. They will also talk to you about things to consider when making treatment decisions.
Symptoms of skin lymphoma can sometimes stay the same for many years. Many cases never develop beyond an early stage of lymphoma. You may not even have symptoms until you notice raised lumps (tumours) on your skin.
You may not need treatment straight away. Instead, your cancer doctor may suggest regular appointments to monitor the lymphoma and check for signs that you need to start treatment. This is called watch and wait. It may also be called active surveillance or active monitoring. It is a way of safely delaying treatment until it is needed.
If you need treatment, your options will depend on the type of skin lymphoma. As skin lymphoma is a rare condition, you may have to travel to another hospital for treatment.
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Treating mycosis fungoides or Sézary syndrome
Early stage MF is often managed by treating the skin directly. For example, you might be given steroid cream. Or you may have phototherapy or radiotherapy.
Drug treatments that work throughout the body are used to treat SS or MF that has spread. These treatments may include chemotherapy or targeted therapy. You might be given tablets or have injections into a vein or under the skin.
A treatment called extra-corporeal photopheresis (ECP) can also treat lymphoma cells in the blood.
We have more detailed information about treatments for MF and SS.
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Treating follicle centre skin lymphoma
Radiotherapy is one of the main treatments for follicle centre skin lymphoma. Your team will explain what your radiotherapy treatment will involve and what to expect.
Surgery is sometimes used to remove small skin tumours. You usually have radiotherapy to the area after surgery. This helps reduce the risk of lymphoma coming back.
If lymphoma is affecting larger areas of skin or has spread to other parts of the body, you will have cancer drug treatments. Rituximab and chlorambucil are 2 drugs used to treat follicle centre skin lymphoma.
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Treating marginal zone skin lymphoma
Rarely, marginal zone lymphoma is caused by an infection called Borrelia burgdorferi. This is the same infection that causes Lyme disease. If tests show you have this infection, you will have antibiotics. For some people, this also reduces the lymphoma and may improve any symptoms.
Most people will have radiotherapy to treat areas of marginal zone skin lymphoma. You can read more about this in our radiotherapy for skin cancer information.
Surgery is sometimes used to remove small skin tumours. You usually have radiotherapy to the area after surgery. This helps reduce the risk of lymphoma coming back.
If lymphoma is affecting larger areas of skin or has spread to other parts of the body, you will have cancer drug treatments. Rituximab and chlorambucil are 2 drugs used to treat marginal zone skin lymphoma.
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Treating primary cutaneous diffuse large B-cell lymphoma
Diffuse large B-cell lymphoma (DLBCL) that starts in the skin is usually treated in the same way as DLBCL that has started in other parts of the body. You can find out more about DLBCL and how it is treated.
You may have some treatments as part of a clinical trial.
Skin care
Skin care is important for all stages and types of skin lymphoma. Your specialist nurse and doctor can give you more advice about looking after your skin. They may suggest moisturising creams or bath lotions to prevent dry skin. These may help your skin feel more comfortable and less itchy and help prevent skin infections.
After skin lymphoma treatment
Follow-up after treatment for skin lymphoma
You will have regular check-ups during and after your treatment. Follow-up appointments are a good time to talk to your cancer doctor or specialist nurse about any concerns you have. Tell them as soon as possible if you have any problems or notice new symptoms between appointments.
We have more information about follow-up care after treatment.
Skin lymphoma is not usually curable. But most types can be treated effectively as a long-term condition. The exception is primary cutaneous diffuse large B-cell lymphoma. This rare type tends to grow more quickly and may need more intensive treatment urgently.
Your team will explain what is likely to happen and how you are likely to be affected. You might have times when you need treatment or help to manage symptoms. At other times, the lymphoma may not change or cause any symptoms.
Well-being and recovery
Looking after yourself can help speed up your recovery after skin lymphoma treatment. Even small lifestyle changes may improve your wellbeing and long term health.
Even if you already have a healthy lifestyle, you may choose to make some positive changes after treatment. We have more information on leading a healthy lifestyle after treatment.
Support for people with skin lymphoma
People often have many different feelings during and after skin lymphoma treatment. If you have finished treatment, you may feel relieved it has ended but worried about what might happen in the future.
It is important to know where to get support or information if you need it. People often need support even some time after skin lymphoma treatment. But sometimes it is difficult to know who to ask for help.
You can:
- ask your GP or someone from your cancer team for advice about support in your area
- find cancer support services near you
- call the Macmillan Support Line for free on 0808 808 00 00.
- chat to our cancer information specialists online.
- visit our non-Hodgkin lymphoma forum to talk to people who have been affected by lymphoma, share your experience, and ask your questions.
You may also find the HOPE programme helpful. The programme is a free 6 week self-management course designed to help you develop techniques and strategies when living with or after cancer. Topics include goal setting, fatigue management, and wellbeing.
You can sign up for the online HOPE programme. Or to find out more about face to face programmes in your area, email ServiceOpsSupport@macmillan.org.uk
Other organisations who offer information and support
- Blood Cancer UK offers support and information to people affected by blood cancers, including lymphoma.
- Lymphoma Action provides high quality information and support for people affected by lymphoma. It provides helpline services and a range of peer support including online support meetings, educational events and webinars. Its website includes TrialsLink, a database of lymphoma clinical trials.
More information and advice
We know cancer can affect you physically, emotionally and financially. We have information and advice about different ways cancer might impact you, such as help with:
About our information
This information has been written, revised and edited by Macmillan Cancer Support’s Cancer Information Development team. It has been reviewed by expert medical and health professionals and people living with cancer.
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References
Below is a sample of the sources used in our lymphoma information. If you would like more information about the sources we use, please contact us at informationproductionteam@macmillan.org.uk
Cwynarski K, Marzolini MAV, Barrington SF, et al. The management of primary mediastinal B-cell lymphoma: a British Society for Haematology Good Practice Paper. Br J Haematol. 2019;185:402-9.
Eyre TA, Bishton MJ, McCulloch R, et al. Diagnosis and management of mantle cell lymphoma: A British Society for Haematology Guideline. Br J Haematol. 2024;204(1):108–26.
Ferreri AJM, Illerhaus G, Doorduijn K, et al., on behalf of the EHA and ESMO Guidelines Committees. Primary central nervous system lymphomas: EHA–ESMO Clinical Practice Guideline for diagnosis, treatment and follow-up. Ann Oncol. 2024;35(6):491-507.
Fox CP, Ahearne MJ, Pettengell R, et al. Guidelines for the management of mature T- and natural killer-cell lymphomas (excluding cutaneous T-cell lymphoma): a British Society for Haematology Guideline. Br J Haematol. 2022;196:507-22.
Fox CP, Chaganti S, McIlroy G, et al. The management of newly diagnosed large B-cell lymphoma: A British Society for Haematology Guideline. Br J Haematol. 2024;204(4):1178–92.
McNamara C, Montoto S, Eyre TA, et al. The investigation and management of follicular lymphoma. Br J Haematol. 2020;191:363-81.
Pratt G, El-Sharkawi D, Kothari J, et al. Diagnosis and management of Waldenström macroglobulinaemia—A British Society for Haematology guideline. Br J Haematol. 2022;197:171–87.
Walewska R, Eyre TA, Barrington S, et al. Guideline for the diagnosis and management of marginal zone lymphomas: A British Society of Haematology Guideline. Br J Haematol. 2024;204(1):86–107.
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