Cutaneous Lymphoma

Cutaneous lymphoma, also known as skin lymphoma, is a cancer that starts in lymphoid tissue in your skin. As part of your lymphatic system, the lymphoid tissue collects excess fluid from your body’s tissues and returns it to your bloodstream to support healthy fluid levels. Lymphomas of the skin are uncommon. They account for only about 4 percent of all non-Hodgkin lymphomas, which is a group of blood cancers that start in lymphocytes, a type of white blood cell, and develop within your body’s lymph system. Cutaneous lymphomas include both B-cell and T-cell lymphomas that involve the skin. 

Types & Stages

There are several types of cutaneous lymphomas. 

• T-cell lymphomas. These are the most common types and are sometimes called cutaneous T-cell lymphomas (CTCLs). They include:
   
  • Mycosis fungoides. Mycosis fungoides is the most common type of cutaneous T-cell lymphoma. It grows slowly. Mycosis fungoides mainly affects the skin. It often causes patches of affected skin.
     
  • Sezary syndrome. Sezary syndrome is a less common type of cutaneous T-cell lymphoma. It grows and expands quickly. Sezary syndrome affects the skin and the blood. In time, it causes a rash over the whole body.
     
• B-cell lymphoma. These lymphomas are sometimes referred to as cutaneous B-cell lymphomas (CBCLs). They’re less common than cutaneous T-cell lymphoma. They include:
   
  • Primary cutaneous follicle center lymphoma. This is the most common B-cell lymphoma of the skin. It tends to grow slowly. The early lesions are groups of red pimples, nodules (lumps) or plaques (raised areas) that form on the scalp, forehead or upper body. Less often, they’re found on the legs. Sometimes the pimples grow into nodules. This type of lymphoma is typically found in middle-aged adults. It tends to respond well to radiation therapy, and most people have an excellent outlook.
     
  • Primary cutaneous marginal zone B-cell lymphoma. This disease is also known as primary cutaneous marginal zone B-cell lymphoproliferative disorder. It tends to grow very slowly and is usually curable. In Europe (but not in the United States), it is sometimes linked to an infection with Borrelia, the germ that causes Lyme disease. This disease can happen at any age, although it tends to happen more often in older adults. It appears as skin lesions that are red to purplish, large pimples, plaques (raised or lowered, flat lesions) or nodules (lumps) on the arms or upper body. There may be only a single lesion, but there can sometimes be a few.
     
  • Primary cutaneous diffuse large B-cell lymphoma, leg type. This is a fast-growing lymphoma that begins as large nodules (lumps), mainly on the lower legs. It happens most often in older people and is more common in women than men. It sometimes spreads to lymph nodes and internal organs, causing serious problems. This type of lymphoma often requires more intensive treatment. The outlook is better if there is only one lesion at the time of diagnosis.

Signs & Symptoms

Signs and symptoms of both cutaneous T-cell lymphoma and cutaneous B-cell lymphoma can differ. 

With cutaneous T-cell lymphoma they include:

• patches of raised or scaly skin that might itch, most often on skin that doesn't get much sun
• patches of skin that look pink, red, brown or gray
• patches of skin that look lighter in color than the skin around them
• lumps that form on the skin and may break open
• lymph nodes that get bigger
• hair loss
• thickened skin on the palms of the hands and soles of the feet
• a rash over all the skin that is scaly and itchy

Signs and symptoms of cutaneous B-cell lymphoma include:

• they may involve one lesion or several
• they may appear on just one part of your body or many areas
• they may be close together or spread out

Primary cutaneous marginal zone B-cell lymphoma may look like:

• flat, thickened areas of skin (plaques)
• large, firm bumps (nodules) or small, pimple-like bumps
• slightly raised skin that’s solid and smooth (papules)
• skin patches that are reddish, blue, purple or brown
• fast-growing bumps on your legs that turn into ulcers

Primary cutaneous diffuse large B-cell lymphoma, leg type, which usually affects your lower legs may cause symptoms, like:

• fever
• night sweats
• unexplained weight loss

Diagnosis

Your doctor will do a physical exam to look over your skin for scaly areas or growths. They may check for other signs of cutaneous T-cell lymphoma, such as swollen lymph nodes. In addition, they may do:

• Blood tests. Blood tests such as a complete blood count can give information about your condition. Sometimes blood tests show cancer cells in the blood. This is more common with the type of cutaneous T-cell lymphoma called Sezary syndrome.
   
• Skin biopsy. A skin biopsy is a procedure to remove cells from the surface of the body so that they can be tested in a lab. The tests can show whether cancer cells are present in the skin. Biopsy types include:
   
  • Punch biopsy. For this, a tool that looks like a tiny round cookie cutter is used to remove a deeper sample of skin. Your doctor rotates the punch biopsy tool on the skin until it cuts through all the layers of the skin. The sample is then removed, and the edges of the biopsy site are stitched together.
     
  • Incisional and excisional biopsies. For these types of biopsies, a surgical knife is used to make an elliptical or circular cut through the full thickness of skin. A wedge or sliver of skin is removed, and the edges of the cut are stitched together. An incisional biopsy removes only part of the tumor. An excisional biopsy removes the entire tumor. 
     
• Imaging tests. If there's concern that the cancer cells have spread to other parts of the body, your doctor might suggest imaging tests. These might include a computerized tomography scan, also called a CT scan, or a positron emission tomography scan, also called a PET scan.

Treatment    

For many skin lymphomas (especially early-stage lymphomas), the first treatment is directed at the skin lesions themselves, while trying to avoid harmful side effects on the rest of the body.  

• Surgery. This is usually in the form of a biopsy and is sometimes part of treatment for certain types of skin lymphomas if there are no more than a few skin lesions and those lesions can be removed completely. If surgery is done, it is often combined with other types of treatment. For example, for cutaneous T-cell lymphoma, treatment depends on the type and can include skin creams, light therapy, radiation therapy and chemotherapy.
   
• Electron beam radiation. The type of radiation used most often for skin lymphomas. The beam of electrons only penetrates as far as the skin, so there are few side effects to other organs and tissues. The main possible side effect of electron beam therapy is a skin reaction similar to a sunburn. The types include:
   
  • Total skin electron beam therapy (TSEBT). For mycosis fungoides and Sezary syndrome covering a large part of the skin, electron beam therapy is sometimes given to the entire body. This is called total skin electron beam therapy (TSEBT).
     
  • High-energy radiation (x-rays or gamma rays). Some thicker lymphomas that aren’t widespread (especially single lesions) are treated with high-energy radiation (like X-rays or gamma rays) instead of electrons.
     
• Phototherapy (UV light therapy). Ultraviolet (UV) light is the higher-energy part of sunlight that causes sunburn and can lead to skin cancer. Phototherapy uses UV light to kill cancer cells in the skin. This is a useful treatment for some skin lymphomas that aren’t very thick, especially if they involve large areas of the skin. The types include:
   
  • Psoralens and UVA (PUVA). When UVA is used, it is combined with drugs called psoralens. This combination is referred to as PUVA.
     
  • Narrowband UVB (NB-UVB). Sometimes described as narrowband UVB or NB-UVB and is given without any extra medicines. It’s generally used for thinner skin lesions.
     
• Topical medicines. Treatment that applies drugs directly to the skin is called topical therapy. It can be very helpful in treating many early skin lymphomas. When a drug is put on your skin, its effects are concentrated on that spot, with much smaller amounts reaching the rest of your body. This can help limit side effects, especially for strong medicines such as some chemotherapy drugs.

Causes & Risk Factors

The risk of cutaneous lymphoma may be higher in:

• Older adults. The condition can happen at any age, but it's more common in people 50 and older.
   
• Gender. The condition is twice as common in men than it is in women. 
   
• Race. Black people have the highest risk of getting cutaneous T-cell lymphoma. Black people also are more likely to get this cancer at an earlier age.
   
• Having a weakened immune system. Skin lymphomas appear to be more common in people with weakened immune systems. This includes people with acquired immunodeficiency syndrome (AIDS), as well as people who’ve had an organ transplant (such as heart, kidney or liver) who must take drugs that suppress their immune system.
   
• Certain infections. Infection with certain viruses or other germs has been suggested as a possible cause of some skin lymphomas. These include: 
   
  • HTLV-1 virus. Infection with the HTLV-1 virus has been linked with the rare adult T-cell leukemia/lymphoma, although most people infected with this virus don’t develop lymphoma. This infection is most often seen in parts of Japan and the Caribbean.
     
  • Epstein-Barr virus (EBV). Infection with Epstein-Barr virus (EBV) has been linked with some types of lymphoma, including extranodal NK/T-cell lymphoma, nasal type. But EBV infection is common, and most people infected with EBV don’t go on to develop lymphoma.
     
  • Borrelia. In parts of Europe, infection with Borrelia, the bacteria that causes Lyme disease, has also been linked with some skin lymphomas. This link has only been reported in a small number of cases. Most people with skin lymphoma have not had Lyme disease, and most people with Lyme disease do not develop lymphoma of the skin.
     
  • Human immunodeficiency virus (HIV). Infection with the human immunodeficiency virus (HIV), the virus that causes AIDS, may increase a person’s risk of skin lymphoma by weakening their immune system.

Screening

With skin self-exams and skin checks by a health care professional like a dermatologist, cutaneous lymphoma can often be found early. When skin cancers are found early, they are likely to be easier to treat.