Skin lymphoma

Non-Hodgkin lymphoma (NHL) can start in the skin. This is called skin lymphoma or cutaneous lymphoma. Most types of skin lymphoma start in T cells and are called T-cell skin lymphomas or cutaneous T-cell lymphomas (CTCLs). Skin lymphomas can also start in the B cells and are called B-cell skin lymphomas or cutaneous B-cell lymphomas (CBCLs).

Sometimes lymphomas start in the lymph nodes or another part of the body and then spread to the skin. Since these did not start in the skin, they are not skin lymphomas. These are other types of B-cell and T-cell lymphoma and are treated differently than skin lymphoma.

The following are specific types of T-cell skin lymphomas and their treatments.

Mycosis fungoides is the most common form of T-cell skin lymphoma. It starts in the skin on areas of the body that are not usually exposed to the sun. It may appear as a scaly, itchy, red rash or raised lumps (called plaques). Mycosis fungoides may be hard to diagnose because it is indolent (slow-growing) and the symptoms can be confused with other skin problems. You may have symptoms for many months or years before mycosis fungoides is diagnosed with a skin biopsy. Mycosis fungoides can progress to tumours on the skin and spread to the lymph nodes, liver, spleen, lungs, blood or bone marrow.

Several treatments may be used for mycosis fungoides at the same time. Treatments may include phototherapy, radiation therapy, immunotherapy, topical medicines (such as corticosteroids), systemic retinoids, systemic chemotherapy, photopheresis, targeted therapy or stem cell transplant.

Sezary syndrome is a very rare and aggressive (fast-growing) form of mycosis fungoides where lymphoma cells spread to the blood and bone marrow. It usually affects most or all of the skin on the body. It can look like a sunburn, and the skin is often red, itchy and peeling. In advanced stages the skin may be very itchy, painful and very sensitive to cold. It is treated like mycosis fungoides.

Primary cutaneous anaplastic large-cell lymphoma (C-ALCL) is indolent (slow-growing) and usually starts as a single lesion or small group of them close together. The lesions can be different sizes, and some may break open and become an open sore.

Primary C-ALCL is treated based on the number of lesions. One lesion or a small area of them may be treated with local therapy such as radiation therapy or surgery. Lesions that occur in multiple locations will be treated with a systemic therapy (such as chemotherapy or retinoids).

Subcutaneous panniculitis-like T-cell lymphoma is a rare type of T-cell skin lymphoma. It starts in the deepest layers of the skin and grows slowly. It can cause lumps to form in the skin, most commonly on the legs. Subcutaneous panniculitis-like T-cell lymphoma may be treated with chemotherapy or radiation.

The following are the most common types of B-cell skin lymphomas and their treatments.

Primary cutaneous follicle-centre lymphoma is the most common type of B-cell skin lymphoma. It tends to grow slowly and appears as groups of red pimples, nodules or plaques on the scalp, forehead or upper body. It may be treated with topical medicines, surgery, chemotherapy, targeted therapy or radiation therapy.

Primary cutaneous marginal zone B-cell lymphoma is a very slow-growing type of B-cell skin lymphoma. It can appear as large red or purple pimples, plaques or bumps on the arms or upper body. It may be treated with topical medicines, surgery, chemotherapy, targeted therapy or radiation therapy.

The following are treatment options for skin lymphomas. Your healthcare team will suggest treatments based on your needs and work with you to develop a treatment plan.

Depending on the type of skin lymphoma you have, you may be offered localized or systemic treatments. Localized treatments are given only to the areas of the skin that are affected. Systemic treatments travel through the whole body. They are used to treat skin lymphoma that has spread to different parts of the body or is too widespread (covering a lot of the skin) for topical therapy.

Since many skin lymphomas are slow growing, you may be offered watchful waiting. The healthcare team will use tests to closely monitor the lymphoma. When symptoms get worse or there are signs that the disease is progressing, they will offer other treatments.

Find out more about watchful waiting.

Surgery is a medical procedure to examine, remove or repair tissue. Surgery, as a treatment for cancer, means removing the tumour or cancerous tissue from your body. It is a localized treatment.

Surgery is most often used to do a skin biopsy to diagnose and determine the type of skin lymphoma.

In some cases, surgery may be used when there are only a few tumours on the skin that can be removed completely. For some skin lymphomas, the only treatment you may need is surgery. Other types of skin lymphomas will need surgery and other treatments as well.

Radiation therapy uses high-energy rays or particles to destroy cancer cells. It is sometimes used to treat skin lymphoma.

Radiation therapy for skin lymphoma is usually given by a machine that directs radiation at the skin (called external radiation therapy). The following are the 2 most common types used.

Involved-site radiation therapy (ISRT) means radiation is given to a small area of skin that is affected by lymphoma.

Total skin electron beam (TSEB) therapy means radiation is given to skin all over the body. It is used if skin lymphoma is widespread. TSEB therapy is usually given only once, but it can sometimes be repeated using lower doses if skin lymphoma comes back after treatment. TSEB therapy can cause a sunburn-like reaction and some people may lose their fingernails, toenails and hair (all over the body).

Find out more about radiation therapy for NHL.

Phototherapy uses either ultraviolet A (UVA) or ultraviolet B (UVB) light to help destroy lymphoma cells. It is a localized treatment. Phototherapy can also be called ultraviolet (UV) light therapy.

PUVA combines the drug psoralen and UVA light. Psoralen is taken as a pill by mouth. About 2 hours later, the skin is exposed to UVA light. Psoralen makes the skin very sensitive to UVA light, which works to activate the drug to destroy lymphoma cells. PUVA treatments are given about 3 times a week until there are no more signs of lymphoma on the skin. They are continued less often as maintenance therapy.

UVB phototherapy uses only UVB light because the skin is sensitive to this type of radiation without psoralen. UVB phototherapy is often used to treat thin patches of lymphoma on the skin. It is usually given 3 to 4 times a week until there are no more signs of lymphoma on the skin (about 30 to 40 treatments). Then it is given less often as maintenance therapy.

Topical medicines are put on the skin. The drug works mostly on the area where it is applied and a small amount will reach the rest of the body. This means that topical medicines usually cause fewer side effects than systemic medicines.

Topical medicines that can be used to treat skin lymphoma include:

• corticosteroids
• chemotherapy drugs, such as carmustine (BiCNU)

Photopheresis is also called extracorporeal photopheresis (ECP). It is a systemic treatment that is sometimes used for Sezary syndrome or other types of CTCL that are progressing.

During photopheresis, blood is removed from the body through a needle. The blood goes into a special machine that takes out the lymphocytes, including the lymphoma cells. The lymphocytes are treated with psoralen and UVA light. After they are treated, the lymphocytes are mixed with the rest of the blood and given back to the person. The procedure takes a few hours. It is usually done 2 days in a row then repeated once about every 4 weeks.

Chemotherapy uses drugs to destroy cancer cells. Systemic chemotherapy is used to treat skin lymphomas that are advanced and have spread to the lymph nodes, blood or other parts of the body. It is also used to treat skin lymphoma that comes back or no longer responds to other treatments.

The most common chemotherapy drugs for skin lymphomas include:

• methotrexate
• gemcitabine
• carmustine
• bendamustine (Treanda, Benvyon, Esamuze)
• fludarabine (Fludara)
• etoposide (Vepesid)
• pralatrexate (Folotyn)
• liposomal doxorubicin (Caelyx)

The most common chemotherapy drug combinations used for skin lymphomas are:

• CHOP – cyclophosphamide (Procytox), doxorubicin, vincristine and prednisone
• GDP – gemcitabine, dexamethasone and cisplatin

Find out more about chemotherapy for NHL.

Targeted therapy uses drugs to target specific molecules (such as proteins) on cancer cells or inside them. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop the growth and spread of cancer cells and limit the harm to normal cells. Targeted therapy may also be called molecular targeted therapy.

Targeted therapies may be used to treat CTCL that doesn't respond to other treatments.The drugs used include:

• vorinostat (Zolinza)
• rituximab (Rituxan and biosimilars)
• brentuximab vedotin (Adcetris)
• mogamulizumab (Poteligeo)
• alemtuzumab (MabCampath)

These targeted therapy drugs may not be covered by all provincial or territorial health plans.

Find out more about targeted therapy for NHL.

Retinoids are drugs similar to vitamin A. Systemic retinoids can be taken as a pill to treat skin lymphoma that is widespread. Retinoids can also be taken as topical medicines, such as a gel, that you can apply on skin lesions.

Retinoids that may be used to treat skin lymphomas include:

• all-trans retinoic acid (ATRA)
• acitretin (Soriatane)
• isotretinoin (Accutane)

A stem cell transplant replaces stem cells. It is sometimes offered for skin lymphomas that don't respond to other treatments.

Find out more about stem cell transplant for NHL.

Talk to your doctor about clinical trials open to people with NHL in Canada. Clinical trials look at new ways to prevent, find and treat cancer. Find out more about clinical trials.