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Skin Lymphoma

Skin lymphoma is a rare type of lymphoma.  It develops when cancerous lymphocytes affect the skin.
This information should not be used to diagnose yourself or in place of a doctor's care.
Diagnosis & staging

In B.C., only about 45 people are diagnosed with this cancer each year.

Skin lymphomas behave very differently from other lymphomas.

Lymphocytes (a type of white blood cell) become cancerous and affect the skin.

Skin lymphoma is hard to diagnose because it looks like other less serious skin conditions. A pathologist can confirm the diagnosis only when the cancer is more advanced and there are enough cancer cells on the skin.

People with skin lymphoma may have symptoms for years before being diagnosed.

People diagnosed with stage 1 or 2 skin lymphoma usually live a long time. For many people, the cancer will not come back (recurrence).

Skin lymphomas are also called T-cell lymphomas (CTCL), mycosis fungoides and Sézary syndrome. Mycosis fungoides is not a fungus infection.

What are the signs and symptoms of skin lymphoma?

  • A chronic rash (rash that does not go away), often itchy. This can be anywhere on your skin.
  • In early stages, it often looks like eczema or psoriasis: red, scaly patches or thicker areas of scaly skin, called plaques.

If you have any signs or symptoms that you are worried about, please talk to your family doctor or nurse practitioner.

How is skin lymphoma diagnosed?

Tests that may help diagnose skin lymphoma include:

  • Physical exam: a careful exam of skin all over your body, looking for lumps, skin lesions, and any other symptoms.
  • Biopsy: a doctor removes a sample of skin. The sample is examined by a specialist doctor (pathologist) under a microscope. This is the only way to diagnose a skin lymphoma.
  • Other tests may be done to see if the cancer has spread:
    • Blood test.
    • Computer tomography (CT) scan:o see the tumour and if the cancer has spread.
    • Lymph node biopsy: a sample of cells is removed from lymph nodes, with a needle or with surgery. A specialist doctor (pathologist) examines the cells for cancer.
For more information on tests used to diagnose cancer, see BC Cancer Library screening and diagnosis pathfinder.

What are the types of skin lymphomas?

There are many types of skin lymphomas.  The type is determined by the type of cell the lymphoma started in and what the lymphoma looks like under a microscope.

Most skin lymphomas are T-cell lymphomas, sometimes called cutaneous T-cell lymphomas (CTCLs). T-cells are white blood cells that have an important role in your immune system (the system that fights infections).

Mycosis fungoides

  • Most common T-cell skin lymphoma.
  • About half of all skin lymphomas.
  • Starts in T-lymphocytes, a type of white blood cell.
  • Usually grows slowly.
  • Is not a fungus infection.

Sézary syndrome

  • Similar to mycosis fungoides but the lymphoma cells are also in the blood or lymph nodes.
  • Often grow and spread faster.
  • Can be harder to treat.

Some skin lymphomas start in B-lymphocytes. These are called cutaneous B-cell lymphomas.  B- lymphocytes are white blood cells that are part of your immune system.

Some types of cutaneous B-cell lymphomas:

  • Primary cutaneous marginal zone B-cell lymphomas.
  • Primary cutaneous follicle centre lymphoma.
  • Primary cutaneous diffuse large B-cell lymphoma, leg type.

What are the stages of skin lymphoma?

Staging describes the cancer. Staging is based on how much cancer is in the body, where it was first diagnosed, if the cancer has spread and where it has spread to.

The stage of the cancer can help your health care team plan your treatment. It can also tell them how your cancer might respond to treatment and the chance that your cancer may come back (recur). 

Mycosis fungoides and Sézary Syndrome staging

Note for all stages: The number of Sézary cells in your blood is measured with a blood test.  The number of cells will be low or high. Please talk to your doctor about your blood test results and how many Sézary cells are in your blood.

Skin lesions but no tumours. Skin lesions cover less than 10% (one tenth) of skin surface.

  • Lymph nodes are not enlarged (larger than they should be).
  • Lymphoma cells have not spread to other organs.
  • Number of Sézary cells in the blood is not high.
 

Skin lesions but no tumours. Skin lesions cover at least 10% of skin surface.

  • Lymph nodes are not enlarged.
  • Lymphoma cells have not spread to other organs.
  • Number of Sézary cells in the blood is not high.
 

Skin lesions but no tumours. Skin lesions cover up to 80% of the skin surface.

  • Lymph nodes are enlarged but patterns of cells do not look very abnormal under a microscope.
  • Lymphoma cells have not spread to other organs.
  • Number of Sézary cells in the blood is not high.
 

At least one of the skin lesions is a tumour that is 1 cm (about half an inch) across or larger.

  • Lymph nodes are either normal or enlarged. Patterns of cells do not look very abnormal under a microscope.
  • Lymphoma cells have not spread to other organs.
  • Number of Sézary cells in the blood is not high.
 

Skin lesions cover at least 80% of the skin surface.

  • Lymph nodes are either normal or enlarged. Patterns of cells do not look very abnormal under a microscope.
  • Lymphoma cells have not spread to other organs.
  • Number of Sézary cells in the blood is low.
 

Skin lesions cover at least 80% of the skin surface.

  • Lymph nodes are either normal or enlarged. Patterns of cells do not look very abnormal under a microscope.
  • Lymphoma cells have not spread to other organs.
  • Number of Sézary cells in the blood is low.
 

Skin lesions cover any amount of the skin surface.

  • Lymph nodes are either normal or enlarged. Patterns of cells look fairly normal under a microscope.
  • Lymphoma cells have not spread to other organs.
  • Number of Sézary cells in the blood is high.
 

Skin lesions cover any amount of the skin surface.

  • Some lymph nodes are enlarged. Patterns of cells look very abnormal under a microscope.
  • Lymphoma cells have not spread to other organs.
  • Sézary cells may or may not be in the blood.
 

Skin lesions cover any amount of the skin surface.

  • Lymph nodes may be normal or abnormal.
  • Lymphoma cells have spread to other organs, such as the liver or spleen.
  • Sézary cells may or may not be in the blood.
 
 

Treatment

What is the treatment for skin lymphoma?

Cancer treatment may be different for each person. It depends on your particular cancer. Your treatment may be different from what is listed here.

External treatment of the skin

  • For stages 1 and 2, a drug may be put on your skin. The main drug used for this is Nitrogen Mustard (Mechlorethamine) ointment.
  • Another treatment option for stages 1 and 2 is ultraviolet light therapy (PUVA). This treatment also uses drug (Psoralen) to make the lymphoma cells sensitive to the light.
  • External treatments for skin lymphomas are not the same as treatments used for other skin cancers.

Radiation therapy (uses high-energy x-rays to kill or shrink cancer)

Systemic therapy (chemotherapy)


What is the follow-up after treatment?

  • Follow-up testing and appointments are based on your type of cancer.
  • After treatment, you may return to the care of your family doctor or specialist for regular follow-up. If you do not have a family doctor, please talk to your BC Cancer health care team.
  • Life after Cancer page has information on the issues that cancer survivors may face.
More information

What causes skin lymphoma and who gets it?

We do not know of any specific causes of skin lymphoma. 

Genetic damage to a person's cells, or immune system suppression (can be caused by a disease such as AIDS), may be risk factors.

Certain skin conditions, such as plaque parapsoriasis or follicular mucinosis, can develop into skin lymphoma.

Statistics on skin lymphoma 

Since this type of cancer is so rare, the statistics are included in the Non-Hodgkin Lymphoma statistics.

Note:  Available statistics do not have information about the inclusion of transgender and gender diverse participants. It is unknown how these statistics apply to transgender and gender diverse people.  Patients are advised to speak with their primary care provider or specialists about their individual considerations and recommendations.

Can I help prevent skin lymphoma?

There are no definite ways to prevent skin lymphomas.

Is there screening for skin lymphoma?

No screening program exists for skin lymphoma.

People with certain skin conditions (plaque parapsoriasis, follicular mucinosis) should have their skin checked often by a doctor or nurse practitioner. 

Where can I find more information?
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SOURCE: Skin Lymphoma ( )
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