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Skin Lymphoma
Revised July 2011
This information should not be used to self-diagnose, or be used in place of a qualified physician’s care.
- Patients with skin lymphoma are treated by members of the BC Cancer Agency’s Lymphoma Tumour Group.
- For healthcare professional information on treating this cancer, please see our Cancer Management Guidelines: Skin Lymphoma
- Some names of skin lymphomas are cutaneous T-cell lymphomas (CTCL), mycosis fungoides and Sézary syndrome.
- This rare type of lymphoma behaves very differently from other lymphomas. The lymphocytes (a kind of white blood cell) become cancerous and affect the skin.
- Mycosis fungoides has nothing to do with a fungus infection.
- This cancer almost always starts on the skin. It can be present on the skin for years before spreading to other parts of the body.
- Skin lymphomas are difficult to diagnose because symptoms are similar to other less serious skin diseases. A pathologist can only confirm the diagnosis when the cancer is advanced and enough lymphoma cells are concentrated on the skin.
- For this reason, even though symptoms may have been noted for years and the patient may even have been under the care of a dermatologist (skin specialist), it is not usually diagnosed in people under 40.
- Patients with stage 1 or 2 usually have a very long life expectancy and some will never relapse after treatment.
What causes it and who gets it?
Listed below are some of the known risk factors for this cancer. Not all of the risk factors below may cause this cancer, but they may be contributing factors.
- No specific causes of skin lymphoma are known. Genetic damage to a person’s cells, or immune system suppression (such as caused by AIDS), may be risk factors.
- Certain specific skin conditions (plaque parapsoriasis, follicular mucinosis) can develop into skin lymphoma.
- Statistics
- Skin lymphomas (T-cell or B-cell, mycosis fungoides, or Sezary syndrome) are very rare.
- In the statistics below, only 5% of those diagnosed with Non-Hodgkin lymphoma will have skin lymphoma. In BC, that might be about 45 people each year.
Can I help to prevent it?
- There are no definite prevention recommendations for skin lymphomas.
- Clinical trials and programs such as the Tumour Tissue Repository hope to learn more about the causes, and prevention, of skin lymphoma in the future.
Screening for this cancer
- A few skin conditions (plaque parapsoriasis, follicular mucinosis) can develop into skin lymphoma. People with these conditions may be checked often, by their doctor, for changes in their disease.
Signs and symptoms
- A chronic rash, often itchy, may appear anywhere on the surface of the body.
- In early stages it appears as either thin red or scaly patches on the skin, or thicker areas of scaly skin (called plaques) that often look like eczema or psoriasis.
Diagnosis
This is a list of some or all of the tests used to diagnose this type of cancer.
- A careful physical exam is done to look for signs of skin lymphoma, such as lumps, skin lesions, and any other symptoms.
- A biopsy is the only way to diagnose skin lymphomas such as Mycosis fungoides.
- For a skin biopsy, affected skin cells are removed by minor surgery in the doctor's office and the sample will be examined under a microscope.
- Other tests to check the extent of the disease may include a blood test, or a CT scan of the abdomen and pelvis.
- If a lymph node biopsy is needed, some cells may be removed with a needle or in surgery, and will be examined under a microscope.
- For more information on all cancer diagnostic tests, see our Recommended Websites, Diagnosis section.
Types and stages
Staging describes the extent of a cancer. The TNM classification system is used as the standard around the world. In general a lower number in each category means a better prognosis. The stage of the cancer is used to plan the treatment.
- T describes the site and size of the main tumour (primary).
- N describes involvement of lymph nodes; NP describes peripheral nodes; NV is visceral nodes.
- M relates to whether the cancer has spread (presence or absence of distant metastases)
- B (for skin lymphoma) describes whether lymphoma cells are circulating in the blood
Stages
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Pre-mycotic stage |
A chronic itchy rash appearing anywhere on the surface of the skin. However it is impossible to confirm a diagnosis. Treatment focuses on relieving the itching with soothing baths, antihistamines, and creams containing steroid hormones. |
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Stage I |
The disease is confined to the skin, forming thin patches or thicker plaques (where the lymphoma cells have spread into the lower levels of the skin; T1 or T2) |
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Stage II |
Similar to Stage I with enlarged lymph nodes, but no lymphoma cells are found if biopsied (N0 or N1-), or forming a solid tumour or lump in the skin (T3). |
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Stage III |
Widespread redness of the skin (erythroderma) (T4). |
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Stage IV |
Any of the above (T4), together with finding lymphoma cells, either in the lymph nodes when a biopsy is done (N1+), or in the blood (B0 or B1) or internal organs or bone marrow (M1). |
To determine the extent of the disease the following procedures may be requested:
- Immunological laboratory tests on blood
- Chest X-ray
- CT scan of the abdomen
- Examination of the blood for lymphoma cells and other blood tests
Types
- Skin lymphomas are often given a name based on the appearance of the lymphoma cells under a microscope.
- Mycosis fungoides is the most common cutaneous T-cell lymphoma (CTCL), which starts in cells called T-lymphocytes (white blood cells).
- Sezary syndrome is like mycosis fungoides, but the lymphoma cells are also found in the blood or the lymph nodes.
- Different kinds of T-cell can become cancerous. Other kinds of T-cell lymphoma which can affect the skin are:
- primary cutaneous anaplastic large cell
- lymphomatoid papulosis
- subcutaneous panniculitis-like T-cell lymphoma
- primary cutaneous peripheral T-cell lymphoma, a rare group including epidermotropic CD8+ cytotoxic, gamma/delta, or CD4+ small/medium sized pleomorphic.
- Cutaneous B-cell lymphoma starts with cells called B-lymphocytes, a white blood cell which makes antibodies. Some B-cell lymphomas are:
- primary cutaneous marginal-zone B-cell lymphoma
- primary cutaneous follicle-centre lymphoma
- primary cutaneous diffuse large B-cell lymphoma, leg type or non-leg.
Treatment
Cancer therapies can be highly individualized – your treatment may differ from what is described below.
External treatment of the skin
- Treatment of stages I and II may be a chemotherapy agent applied to the skin. For skin lymphomas, the main chemotherapy drug used on the skin is Nitrogen Mustard (Mechlorethamine) ointment.
- Another treatment option for stages I or II can be ultraviolet light therapy (PUVA), using a drug (Psoralen) to make the lymphoma cells sensitive to the light.
- External treatments for lymphomas of the skin are not the same as treatments used on other skin cancers.
Surgery
- Surgery is not used to treat skin lymphomas, although a surgical biopsy (sample) of the area might be used to understand the stage of it.
Radiation Therapy
- Radiation therapy of the involved part of the skin is usually very effective.
- Radiation therapy to the whole skin surface may be used if external skin treatments are not effective.
Chemotherapy
- Chemotherapy may be recommended if the skin lymphoma has spread. Drugs used for these lymphomas in the skin are not the same as drugs used for other skin cancers.
- Some chemotherapy drugs are given as pills, to be taken by mouth. Others are given intravenously, through a needle in a vein (IV). Useful drugs include Acitretin, Chlorambucil, or Gemcitabine.
- Radiation therapy to affected areas of the skin may be used at the same time.
Follow-up after treatment
- Guidelines for follow-up after treatment has ended have been developed by the BC Cancer Agency and are listed on our website.
- You will be returned to the care of your family doctor or specialist for regular follow-up
- Follow-up testing is based on your type of cancer and your individual circumstances.
- The BCCA Survivorship Research Centre focusses on the issues that cancer survivors can face.
Coping with cancer
The Coping with Cancer section of our website is a joint project among different BC Cancer Agency departments and programs. This website section provides information and links that can help cancer patients with the physical, emotional, psychological and practical aspects of cancer care. Each cancer experience is different, but in one way or another, many cancer patients share the same needs.
The effects of cancer and its treatment can present unique challenges: from practical concerns like money and housing, to emotional concerns like anxiety and grief. If you need support with the practical and emotional impacts of cancer, or in managing symptoms and side effects you can use the information in Coping with Cancer to connect to these resources.
Search our library catalogue
- The BC Cancer Agency Library has many resources about cancer, coping, talking to children, etc. Please visit the Library in your Centre, call a librarian, or visit the Library online to see the many resources available.
- Automatically get a bibliography of lymphoma books, videos and other items available through our library. Many of these items will have a section or chapter about skin lymphomas.
Recommended websites
The BC Cancer Agency has selected and evaluated useful websites for further reading.
Can I help with research at BC Cancer Agency?
BC Cancer Agency patients are very helpful when it comes to the fight against cancer. Here are a few ways that you can help:
This information is awaiting Tumour Group approval.
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