Skin (cutaneous) T-cell lymphoma

Skin (cutaneous) lymphomas are non-Hodgkin lymphomas that start in the skin. There are several different types. This page is about the most common group, cutaneous T-cell lymphomas. A lymphoma that starts somewhere else, for example in the lymph nodes, and then spreads to the skin is not a skin lymphoma. If you have a lymphoma that has spread to the skin, our information on non-cutaneous lymphomas will be more relevant for you.

On this page

What is skin lymphoma?

Causes

Symptoms

Diagnosis and staging

Outlook

Mycosis fungoides

Sézary syndrome

Primary cutaneous CD30-positive lymphoproliferative disorders (ALCL and lymphomatoid papulosis)

Rare types of cutaneous T-cell lymphoma

Research and clinical trials

Check-ups

What is skin lymphoma?

Lymphoma is a cancer that starts in white blood cells called lymphocytes, which are part of the immune system. Lymphocytes are normally found in lymph nodes (glands). They are also found in lymphoid tissues, for example, in the spleen, as well as the gut and the skin.

If lymphocytes start to grow out of control or don’t die after their normal lifespan, they can build up and form a cancerous collection of cells – this is a lymphoma. If lymphoma starts in the lymphocytes in the skin it is called a ‘cutaneous’ lymphoma, which means a lymphoma ‘of the skin’.

Skin lymphomas are rare cancers. Around 10 out of every million people develop a skin lymphoma each year in the UK. That is about 1 in 40 of all the non-Hodgkin lymphomas diagnosed.

There are 2 types of lymphoma: Hodgkin lymphoma and non-Hodgkin lymphoma. Within each of these types, there are many sub-types. All skin lymphomas are non-Hodgkin lymphomas.

There are 2 types of lymphocyte: T lymphocytes (T cells) and B lymphocytes (B cells). Skin lymphomas can develop from either T cells or B cells.

  • Cutaneous T-cell lymphomas (CTCL) are the most common types of skin lymphoma.  More than 3 out of every 4 skin lymphomas diagnosed are CTCLs. They often appear as eczema-like skin rashes and can affect widespread parts of the body. There are different subtypes of CTCL. The most common type is mycosis fungoides.
  • Cutaneous B-cell lymphoma (CBCL) more commonly causes lumps in the skin, usually in 1 or 2 areas of the body. Fewer than a quarter of skin lymphoma cases are CBCLs.

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Causes

Scientists don’t know exactly what causes skin lymphoma. Research has shown that:

  • it isn’t caused by anything you’ve done
  • it isn’t passed down through families (inherited)
  • you can’t catch it or pass it on.

Scientists know from research that the lymphocytes become cancerous because of changes in the chemical coding (DNA) of their genes. These changes are not inherited from your parents – they occur in the lymphocyte cells after your birth. Scientists don’t really understand why these changes happen, but age is probably a factor. Most skin lymphomas occur in people over 50.

Although scientists don’t know exactly what causes skin lymphoma, there are a few things that make them slightly more likely to develop. These are known as ‘risk factors’. It is important to realise that most people with these risk factors do not develop lymphoma.

Some risk factors apply to many types of lymphoma. People with immune system problems are more likely to develop lymphoma than are people with healthy immune systems. Immune system problems can develop following an organ transplant and resulting from taking drugs to prevent rejection. Lymphoma is also more common in people with acquired immune deficiency syndrome (AIDS) and in people with certain rare inherited diseases of the immune system, such as ataxia telangiectasia.

Some other risk factors for developing a CTCL include: 

  • Sex – many types of skin lymphomas are more common in men.
  • Race – in the United States skin lymphoma is more common in Afro-Caribbean people than it is in the white population, but scientists don’t know why.
  • Human lymphotropic virus 1 (HTLV-1) infection – the virus occurs in some parts of the world including the Caribbean and parts of Asia, eg Japan.

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Symptoms

Most types of CTCL begin as flat, red patches on the skin.

Patches of mycosis fungoides showing areas of dry, red skin
Figure: Patches of mycosis fungoides, showing patches of dry, red skin

The patches may be itchy, but in some people they are not irritating at all (unlike patches of eczema). These patches tend to be dry and sometimes scaly. They can look like more common skin conditions, such as dermatitis, eczema or psoriasis. Patches are most common on the buttocks or torso, but they can occur anywhere.

CTCL can show up as patches of altered skin colour, particularly in non-white skin. These patches might be lighter (hypopigmented) or darker (hyperpigmented) in colour than normal skin. They may also be areas with a net-like pattern of pigmentation (poikiloderma).

If areas of skin become harder and thicker but still quite flattened, they are called plaques. In many people, the skin lymphoma never develops beyond patches and plaques.

Papules can also appear. These are small, raised solid areas of skin that can look like a rash. 

Plaques of mycosis fungoides    
Figure: Plaques of mycosis fungoides
Purple-red patches with papules
Figure: Purple-red patches with papules

Some people develop larger swellings in the skin, called nodules or tumours. These can ulcerate (break down) and become infected. You may only have 1 or 2 of these tumours, but in some skin lymphomas there can be several.

 A tumour that has ulcerated and scabbed over
Figure: A tumour that has ulcerated and scabbed over

In some people, large areas of the skin become reddened, thickened, swollen and sore. This is erythroderma. The skin on the palms of the hands and soles of the feet can also become thickened and may crack.

erythroderma
Figure: Erythroderma

You may also have swollen lymph nodes in your neck, armpits or groin. They may be inflamed as a reaction to the nearby skin irritation or because they contain abnormal lymphoma cells.

If abnormal lymphoma cells (sometimes called Sézary cells) are circulating in your bloodstream, your whole body can feel very itchy. If you have large numbers of these cells, this can lower your immunity and make you more prone to infections.

A small number of people with a CTCL have more general symptoms such as unexplained weight loss, fevers or night sweats.

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Diagnosis 

You are most likely to go to your GP if you have red or itchy patches of skin or if you have lumps anywhere. Many skin lymphomas look like more common skin conditions such as eczema or psoriasis. Many of them also develop very slowly, some over as long as 10–40 years. It may take a long time for your GP to rule out other conditions and refer you to a specialist. This might be a specialist in skin diseases (dermatologist) or a specialist in diseases of the blood and lymphatic system (haematologist).

At the hospital, the specialist will ask how and when the skin problem developed and how it affects you. They will examine you, looking carefully at skin patches or lumps. A medical photographer might take pictures of your skin. The specialist will also ask about your general health and about any other symptoms you’ve had, such as weight loss or fevers.

Your doctor might suspect what the problem is, but will have to confirm the diagnosis with a skin biopsy. In a biopsy, the doctor numbs an area of affected skin with a local anaesthetic and removes a small piece of the skin. The sample is then examined under a microscope and sent for specialised tests to look at the cells and their genes and proteins in detail. These tests sometimes have to be done in a laboratory at another centre. Results of the biopsy can take 2–3 weeks to come back.

Diagnosing skin lymphoma is not always straightforward, even for a specialist. You might need further skin biopsies over the following few weeks or months. In some people, the skin rash does not look typical of lymphoma. In this case, you may need several biopsies taken over a few years before you are diagnosed. This can be a frustrating and anxious time. It is important that the doctors make an accurate diagnosis and find out as much as possible about your skin condition so that you can have the most suitable treatment.

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Tests and scans

The history of how and when your skin problem developed, the examination of your skin and the results of skin biopsies help your medical team to diagnose your lymphoma. To find out more about the lymphoma and how it is affecting your body, you also need to have a full physical examination and blood tests. These tests are needed for ‘staging’ of the lymphoma.

When examining you, the doctor will feel for enlarged lymph nodes in your neck, under your arms and in your groin. You won’t need any internal examinations. The blood tests will include blood cell counts and measurements of levels of some chemical substances found in the blood, including lactate dehydrogenase (LDH). This is an enzyme in your body that is used in the process of turning sugar into energy.

Further tests depend on exactly which type of lymphoma you have and on your general health. If you have the most common T-cell skin lymphoma, mycosis fungoides, and your physical examination and blood tests are normal, you only need a chest X-ray.

Scans for T-cell skin lymphomas are not done as often as they are for other types of non-Hodgkin lymphoma. You may have a scan if other investigations suggest there are lymphoma cells in your blood or lymph nodes (glands).

The most common type of scan for skin lymphoma is a computed tomography (CT) scan of your chest, abdomen and pelvis (area between your hip bones). Some people may have another scan called positron-emission tomography (PET), which may be combined with CT into a PET/CT scan. These scans capture your internal organs in great detail. You usually have them as an outpatient and they can take anything from 30 minutes to 2 hours. You may have to go to a larger medical centre than your local hospital. Your doctor should give you all the details you need.

A few people with suspected skin lymphoma have a bone marrow biopsy. A bone marrow biopsy involves taking a small sample of the bone marrow (the spongy tissue in the centre of some of the large bones of the body where blood cells are made) from your hip bone with a needle. The doctor numbs the skin over the bone with a local anaesthetic first.The sample is then examined under a microscope to see if it contains lymphoma cells. You have painkillers to help with any discomfort after the procedure.

If you have enlarged lymph nodes, you may need a lymph node biopsy. A lymph node biopsy involves having a node removed under a local or general anaesthetic. You may hear this called an ‘excision biopsy’. The node is then sent to the laboratory to be examined under a microscope.

Some people may also have a fine needle aspirate (FNA) of a lymph node. This is where a fine needle is used to remove some cells from the enlarged lymph node without it being removed. An FNA is sometimes done before you are referred to the specialist clinic. However, after an FNA, you are still likely have a lymph node biopsy as a FNA only samples some of the cells in the lymph node. This means abnormal cells might be missed.

All these tests are done to find out which parts of your body the lymphoma is affecting. They are also done to make sure that the lymphoma definitely started in the skin rather than spread there from somewhere else. This is important. Lymphomas that start inside the body behave differently to skin lymphomas and need different treatment. Once all the results are back, your medical team can decide on the best course of treatment for you.

The appearance of your skin, together with the physical examination and other test results usually provide:

  • a diagnosis of the exact type of skin lymphoma you have – whether it is a T-cell or a B-cell skin lymphoma and exactly which type
  • information on whether the lymphoma is a slow-growing (low-grade or ‘indolent’) type or faster-growing (high-grade) type
  • an indication of the stage of the disease.

The type, grade and stage of a lymphoma help your doctors to predict how it is likely to behave in the future and decide how best to treat it.

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Staging

The stage of the lymphoma describes how much of your body has been affected by lymphoma. Most T-cell lymphomas of the skin are diagnosed at an early stage. The staging system used depends on the type of CTCL you have.

Stages of mycosis fungoides and Sézary syndrome

Mycosis fungoides and Sézary syndrome are closely related. However, most people with Sézary syndrome develop it without having had any milder form of skin lymphoma previously. Very few people with early-stage mycosis fungoides go on to develop Sézary syndrome.

To find out the stage of your lymphoma, your doctors check:

  • how your skin is affected – whether there are patches, plaques or tumours
  • how much of your skin is affected
  • whether the lymphoma is in any of your lymph nodes
  • whether the lymphoma is in any of your internal organs
  • whether you have abnormal lymphoma cells (Sézary cells) in your blood.

Like most cancers, there are 4 main stages for these types of CTCL.

Stage 1

The lymphoma only affects the skin (patches or plaques):

  • stage 1A means that less than a tenth of the skin is affected
  • stage 1B means that a tenth or more of the skin is affected.

Stage 2    

  • Stage 2A means that there are patches or plaques on the skin and the lymph nodes are enlarged but they do not contain abnormal lymphoma cells.
  • Stage 2B means that there are one or more raised lumps or tumours in the skin and the lymph nodes may or may not be enlarged but do not contain lymphoma cells.

Stage 3    

Four-fifths or more of the skin is affected, with generalised redness, swelling, itching and sometimes pain (erythroderma).The lymph nodes can be enlarged, but don’t contain abnormal lymphoma cells. In addition:

  • stage 3A means there are few or no lymphoma cells in the bloodstream (erythrodermic mycosis fungoides)
  • stage 3B means there are moderate numbers of lymphoma cells in the bloodstream (Sézary syndrome).

Stage 4    

In addition to skin problems:

  • stage 4A means there are numerous abnormal lymphoma cells in the bloodstream (Sézary syndrome) or the lymph nodes contain lymphoma cells
  • stage 4B means there is lymphoma in other organs.

You may also see the stages referred to as Roman numerals: I, II, III or IV.

‘Early’ stage means anything up to 2A. Most people have this stage of skin lymphoma when they are diagnosed. A few people have more advanced disease (stages 2B, 3 and 4). Very rarely, the blood is affected at diagnosis (stages 3B or 4A, also called ‘Sézary syndrome’).

Staging of other CTCLs

Different staging systems are used for other, rarer types of CTCL. These systems are usually based on the TNM staging. TNM stands for tumour, node, metastasis. This is a way of recording cancer stages and describes:

  • how many areas of changed skin there are, how big they are and where they are (shown by a ‘T’ and a number between 1 and 3)
  • how many lymph nodes are involved (if any) and which ones are involved (shown by an ‘N’ and a number between 0 and 3)
  • whether any other parts of the body are involved (ie parts that are not skin or lymph nodes, shown by an ‘M’ and either 0 or 1).

The TNM system is useful because it is detailed and can flag up changes in stage over time. This can help your doctors to monitor your condition and help determine the best treatment for you.

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Grading

Slow-growing lymphomas are described as ‘low-grade’ and faster-growing lymphomas as ‘high-grade’. Most skin lymphomas are slow-growing. The table below divides the different types of CTCL into the ones that are slow-growing and those that grow more rapidly. Knowing how fast the lymphoma is developing is important in choosing the best treatment and in deciding how soon your treatment should start.

Slow-growing types of CTCL  

Faster growing types of CTCL

* Rare types of CTCL that don’t fit into one of the defined types are grouped as primary cutaneous peripheral T-cell lymphoma, unspecified.

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Outlook

Skin lymphomas are considered to be incurable. In most cases, they are chronic (long-term) conditions that are not life-threatening. However, the prognosis (outlook) depends on the type of CTCL, your age and general health, and on how much of your skin is affected.

Most skin lymphomas never develop beyond early stages. They are often diagnosed early, grow slowly and respond well to treatment. Any skin problems they cause come and go and only need treatment some of the time.

If less than a tenth of your skin is affected (stage 1A), this is not a life-threatening condition. It is rare for the skin of people with stage 1A of the most common type of cutaneous T-cell lymphoma (mycosis fungoides) to remain completely and permanently clear after treatment. However, as long as the lymphoma is controlled reasonably well, it is unlikely to spread beyond the skin.

If more than a tenth of your skin is involved, or the lymphoma has spread to lymph nodes or other organs (stage 1B) by the time treatment starts, the disease may come back more quickly after treatment. Should it come back, it can often be controlled for many years with treatment.

Some skin lymphomas are faster-growing and more aggressive in their behaviour from the outset. These develop more rapidly and need more intensive treatment more urgently.

Some slow-growing skin lymphomas occasionally change into a faster-growing type. This is called transformation. Your medical team will check for it when you go to the clinic. Should this happen, you will need more intensive treatment.

The following sections contain more specific information about the different types of CTCL.

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Mycosis fungoides

Almost 2 out of every 3 people with a T-cell skin lymphoma have mycosis fungoides, which is the most common skin lymphoma overall. It is a slow-growing (low-grade) lymphoma that develops over many years or even decades. It is rarely life-threatening. It usually has little effect on quality of life.

Mycosis fungoides got its name because the spots it’s associated with were thought to resemble a mushroom-like fungal disease. It was first described at the beginning of the 19th century. It is not caused by a fungus at all, but the name has stuck.

Scientists don’t know what causes mycosis fungoides. It occurs most commonly in people aged between 40 and 60 and affects twice as many men as women.

About 3 out of every 4 people have early-stage disease when they are first diagnosed. Most people have the classic type, but there are several rarer forms.

Classic mycosis fungoides

Classic mycosis fungoides starts as irregularly shaped, oval or ring-like (annular), dry or scaly patches. They are usually flat and either discoloured or pale. They can disappear spontaneously, stay the same size or slowly enlarge. They are most common on the torso or buttocks but can occur anywhere. They are often mistaken for more common skin conditions, such as eczema or psoriasis, sometimes for many years.

Sometimes similar, but thicker and slightly raised areas of skin develop, called ‘plaques’. They can be itchy and can sometimes ulcerate (break down). The most common places for them to appear are the buttocks and folds of skin. You may lose hair in affected areas.

Mycosis fungoides rarely develops beyond the early patch and plaque stage. In a small proportion of people, raised lumps appear on the skin. These tumours can ulcerate or weep and they can be painful.

In very few people the skin can become red, thickened and sore all over. This is called erythroderma. If this happens, it is possible that there are some lymphoma cells in the lymph nodes or blood too. Mycosis fungoides does not usually involve other internal organs or the bone marrow.

Treatment for classic mycosis fungoides

Mycosis fungoides can be very well controlled with treatment, but it has a tendency to come back when treatment is stopped. A few people need no treatment at all at first. A good skincare regime with regular use of moisturisers helps to prevent dryness and irritation. If you have stage 1 disease, this may be all you need.

If larger areas of your skin are affected, or if you have troublesome itching, you may have treatments applied directly to the skin. Topical treatments include steroid creams, light therapy (PUVA or narrow band UVB treatment) or chemotherapy drugs in a cream that you apply to your skin (such as carmustine [BiCNU®]). If you have skin tumours, you may have radiotherapy.

If you have disease in your lymph nodes, or more extensive plaques and tumours, or if the topical treatments have not worked in early-stage disease, you may have:

If mycosis fungoides doesn’t respond to these treatments, or if you have more advanced disease diagnosed, you may have:

  • oral chemotherapy drugs (usually tablets), such as methotrexate or etoposide
  • a different oral retinoid drug used on its own, such as bexarotene (Targretin®)
  • interferon alpha by subcutaneous injection if not previously used with PUVA
  • single-agent intravenous (into a vein) therapy, which may be chemotherapy, for example gemcitabine, fludarabine, deoxycoformycin or liposomal doxorubicin (Caelyx®), or a monoclonal antibody like alemtuzumab (Campath®)
  • a chemotherapy regimen (combination of drugs), such as CHOP, which is often used to treat people with other types of lymphoma
  • a combination of chlorambucil (a type of chemotherapy) tablets and steroid tablets
  • a targeted drug that is not yet licensed in the UK but may be available in a clinical trial or through an individual funding request, such as the histone deacetylase (HDAC) inhibitor, vorinostat.

For erythroderma, you may have extracorporeal photopheresis (ECP). This is a type of light treatment where your blood (rather than your skin) is treated with ultraviolet A light. The blood is then transfused back into you.

Young, fit patients with advanced mycosis fungoides may have a stem cell transplant using cells from a donor (an allogeneic transplant).

Rarer forms of mycosis fungoides

Apart from the classic form of mycosis fungoides there are 3 other, rarer forms that behave slightly differently and look different under a microscope.

  • Folliculotropic mycosis fungoides affects hair follicles in particular. It commonly affects the head and neck and can cause hair loss. You may have just one patch, plaque or tumour but most people have several. You may have small cysts or blocked pores. These are sometimes called ‘comedome’ (whiteheads) or ‘milia’ (milk spots) as they look like white bumps on the skin. Topical therapies, such as PUVA and chemotherapy ointments, don’t work well for this type of skin lymphoma. Your doctor may suggest total-skin electron beam therapy, PUVA combined with retinoid drugs, interferon or radiotherapy.
  • Pagetoid reticulosis (Woringer–Kolopp disease) usually shows up as a single scaly plaque, often on an arm or leg. It never spreads beyond the skin. You may have surgery or a low dose of radiotherapy to treat it.
  • Granulomatous slack skin (GSS) is an extremely rare form of mycosis fungoides. Loose folds of skin develop in the armpits and groin. There is no agreed standard treatment for this type of CTCL. Your doctor may suggest surgery, radiotherapy, PUVA, steroid creams or interferon.

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Sézary syndrome

About 1 in 20 people with CTCL has Sézary syndrome, which is similar to mycosis fungoides but affects the blood as well as the skin. Most people with Sézary syndrome develop it without having had any milder form of skin lymphoma beforehand. Very few people with early-stage mycosis fungoides go on to develop Sézary syndrome. Sézary syndrome is diagnosed if you have:

  • large areas of the skin that are bright red, thickened, swollen and sore (erythroderma)
  • large numbers of abnormal lymphocytes (Sézary cells) in your blood
  • abnormal lymphocytes in your lymph nodes, which may be enlarged.

Your skin can be extremely itchy. You may also have:

  • hair loss
  • thickening of the skin of the palms of the hands and the soles of the feet
  • exposure of the inside surface of the lower eyelid (ectropion).

A large number of lymphoma cells circulating in the bloodstream means that the immune system doesn’t work as well as it should. This can make you more prone to infection.

Treatment for Sézary syndrome

Sézary syndrome affects the whole body, not just the skin. It needs to be treated with systemic therapy, which is treatment that reaches all parts of the body. The first choice of treatment is usually extracorporeal photopheresis (ECP).

Other treatments include low-dose methotrexate, bexarotene (Targretin®), interferon alpha, the antibody alemtuzumab (Campath®) and combination chemotherapy (for example CHOP) or a combination of chlorambucil tablets and steroid tablets. You may have a topical treatment such as PUVA as well as other treatments. Should you get an infection, you will need antibiotics.

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Primary cutaneous CD30-positive lymphoproliferative disorders

There are 2 main types of these disorders:

Together they make up almost a third of all diagnosed T-cell skin lymphomas. In both conditions, abnormal lymphocytes have a protein CD30 on their surface.

Both of these conditions can be well-managed and are not normally life-threatening.

Primary cutaneous anaplastic large-cell lymphoma (ALCL)

Primary cutaneous ALCL is most common in people in their 50s and 60s, but can occasionally occur in children. It is 2–3 times more common in men than women. It usually appears as a single tumour on the skin, but you may have a group of tumours in one area. Tumours usually appear on the torso, arm or leg and can ulcerate (break down). They can go away completely without any treatment and rarely spread beyond the skin.

If you need treatment for a single tumour, you are most likely to have surgery or radiotherapy. If you have several tumours in one area, you are most likely to have radiotherapy. If you have many tumours, you may have chemotherapy with methotrexate, or immunotherapy. If the disease has spread to the nearby lymph nodes, you may have a combination of chemotherapy drugs.

Lymphomatoid papulosis (LyP)

This is a very-slow growing skin lymphoma. It tends to come and go and you may not need any treatment at all. The average age at diagnosis is about 45 and it is more common in men. It usually shows up as crops of red spots (papules) or bigger lumps (nodules), which appear over a period of a few days, usually on the torso, arm or leg. Tumours may ulcerate (break down) in the middle. They usually heal over 3–12 weeks, but they can take longer to heal or may persist. You may have superficial scars left behind after the ulcerations have healed.

About 1 in 20 people with this condition develop another type of lymphoma as well, such as cutaneous ALCL, mycosis fungoides or Hodgkin lymphoma.

There is no treatment that can get rid of LyP or stop it appearing. Several topical treatments (applied directly to the skin) can help if the nodules are troublesome, including PUVA and steroid creams. If you have very frequent or severe attacks, your doctor may suggest treatment with a low dose of the chemotherapy drug methotrexate.

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Rare types of cutaneous T-cell lymphoma

There are several rare types of CTCL.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is slow-growing and can occur at any age. It is slightly more common in women. It starts in the fatty layer of the skin, just below the surface. You may have one or more plaques or nodules, often on the legs. You may also have other more general symptoms, such as fevers, low blood counts and weight loss.

This condition responds very well to steroid tablets, which may be the only treatment you need. If you do need more treatment, you may have local radiotherapy (only to the affected area) or chemotherapy with doxorubicin. If your SPTCL is faster growing, your doctor may suggest a combination of chemotherapy drugs such as CHOP or even a stem cell transplant.

Primary cutaneous CD4-positive small/medium T-cell lymphoma is a slow-growing lymphoma with a good prognosis (outlook). It usually appears as a single plaque or nodule on the face, neck or upper torso. Treatment is usually to remove the plaque or nodule surgically or attack it with radiotherapy. If the lymphoma is more widespread, your doctor may suggest drug treatment with either a chemotherapy drug called cyclophosphamide or an immunotherapy drug, interferon alpha. Localised disease of this type may be re-classified as a lymphoproliferative disorder rather than a cancerous condition in the future because of its slow-growing nature and good outlook.

Primary cutaneous gamma/delta T-cell lymphoma is a faster-growing type of skin lymphoma that usually occurs in adults. It most commonly shows up as patches and plaques on the arms or legs. You may also have night sweats, fevers and weight loss. Some people develop low blood counts and an enlarged liver and spleen. Your doctor is most likely to suggest treatment with a combination of chemotherapy drugs, or, in some circumstances, a stem cell transplant.

Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma is a faster growing lymphoma that occurs mainly in adults. It appears as widespread spots (papules), plaques and tumours on the skin. Affected areas may ulcerate. It can also affect the lining of the mouth. Your doctor is most likely to suggest treatment with a combination of chemotherapy drugs, or, in some circumstances, a stem cell transplant.

Extranodal NK/T-cell lymphoma, nasal type, is a rare form of lymphoma that is sometimes seen in the skin. It can also start elsewhere but involve the skin. This is a fast-growing type that is most common in Asia and Central and South America. People with this type of lymphoma usually test positive for Epstein-Barr virus (EBV). In most cases, this type of lymphoma is treated with systemic (whole body) chemotherapy.

Read our section on treatment for skin lymphoma for more information on the types of treatment you might have.

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Research and clinical trials

Researchers are continually trying to find out which treatment, or combination of treatments, works best for skin lymphomas. Your doctor may ask if you would like to take part in a clinical trial. Clinical trials allow new treatments to be evaluated and compared with more established ones. Studying treatments is the only way that new and, hopefully, better treatments can become available. 

Find out more about clinical trials and search for a clinical trial that might be suitable for you at Lymphoma TrialsLink.

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Check-ups

How often you have check-ups depends on what type of skin lymphoma you have, the stage of your disease and how it is responding to treatment. You will probably only see your specialist every 6 months if:

  • you have a slow-growing lymphoma at an early stage
  • your doctor is monitoring your condition and you are not having active treatment
  • your condition is stable (unchanging) or in remission (no evidence of the disease) after treatment.

You may see your doctor as often as every 4–6 weeks if:

  • your lymphoma is a faster-growing type,
  • your lymphoma is continuing to grow
  • you are on treatment that means you need close monitoring.

At the clinic, your doctor will ask about your symptoms and examine you. Let them know if there are any symptoms that have been troubling you, any changes to your skin or new swellings that have developed. You will only occasionally need blood tests, scans or further biopsies.

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Further reading

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