Cutaneous Lymphoma

Cutaneous lymphoma – Cancers of lymphocytes (white blood cells) that primarily involve the skin. Cutaneous lymphomas are a distinct subset of non-Hodgkin’s lymphoma that is often indolent.

Risk Factors

While most people with lymphoma of the skin may have some factors that make them more likely to get this disease (such as their age or gender), in most people there is no clear cause of the lymphoma.
  • Age: Age is an important risk factor for this disease, with most cases occurring in people in their 50s and 60s. But some types of skin lymphoma can appear in younger people, even in children.
  • Gender and race: Most (but not all) types of skin lymphoma are more common in men than in women. Most also tend to be more common in African-Americans than in whites. The reasons for this are not known.
  • Weakened immune system: Skin lymphomas may be more common in people with acquired immunodeficiency syndrome (AIDS), who have a weakened immune system. They may also be more common in people who have had an organ transplant such as a heart, kidney or liver transplant. These people must take drugs that suppress their immune system, which may raise the risk of skin lymphoma (or lymphomas in other parts of the body).
  • Infections: Infection with the human immunodeficiency virus (HIV), the virus that causes AIDS, may increase a person’s risk of skin lymphoma. Other infections such as Epstein-Barr virus (EBV), HTLV-1 virus and Lyme disease have been rarely associated with the disease. However, just because you may have this infection, it does not mean that you will develop lymphoma.

Signs and Symptoms

Any of a group of T-cell non-Hodgkin lymphomas that begins in the skin as an itchy, red rash that can thicken or form a tumor. The most common types are Lymphomas of the skin can be seen and felt. They can appear as:
  • Papules (small, pimple-like lesions)
  • Patches (flat lesions)
  • Plaques (thick, raised or lowered lesions)
  • Nodules or tumors (larger lumps or bumps under the skin)
The lesions are often itchy, scaly, and red to purple in color. The lymphoma might show up as more than one type of lesion and on different parts of the skin (often in areas not exposed to the sun). Some skin lymphomas appear as a rash over some or most of the body (known as erythroderma). Sometimes larger lesions can break open (ulcerate). Along with skin problems, in rare cases lymphoma of the skin can cause general symptoms, such as:
  • Unexplained weight loss
  • Fever
  • Profuse sweating (enough to soak clothing), particularly at night
  • Severe itchinessmycosis fungoides and Sézary syndrome.

Diagnosis

A biopsy is a procedure in which a doctor removes a sample of body tissue for viewing under a microscope or other lab tests. A biopsy is needed to diagnose lymphoma of the skin.

Skin Biopsies

There are several types of skin biopsies, and the doctor’s choice is based on each person’s situation. Usually a skin biopsy is done by a dermatologist.
  • Punch biopsy: For a punch biopsy, the doctor uses a tool that looks like a tiny round cookie cutter (usually a little more than 1/8 inch across). Once the skin is numbed with a local anesthetic, the doctor rotates the punch biopsy tool on the surface of the skin until it cuts through all the layers of the skin. Often the biopsy site is closed with a stitch.
  • Incisional and excisional skin biopsies: For these types of biopsies, a surgical knife is used to cut through the full thickness of skin. An incisional biopsy removes only part of the tumor, while an excisional biopsy removes the entire tumor. The piece of skin is removed for testing, and the edges of the cut are sewn together. These biopsies are usually done using a local anesthetic (numbing medicine).
Regardless of the type of skin biopsy, once the samples are removed, they are sent to a doctor called a pathologist, who will look at them under a microscope and might do other tests on them (see below). Many of the more common forms of skin cancer (and other skin diseases) can be diagnosed just by looking at the biopsy samples under a microscope. But diagnosing and classifying lymphomas of the skin often requires one or more special lab tests (see below). Diagnosing some forms of skin lymphoma can be very challenging. Sometimes, especially if the diagnosis is unclear, the skin samples may need to be sent to a dermatopathologist, a dermatologist or a pathologist with additional training in diagnosing skin samples. Even with this expertise, in some cases several biopsies may be needed over a period of time before the diagnosis is confirmed.

Lymph Node Biopsies

Skin lymphomas often spread to lymph nodes, so your doctor may recommend a lymph node biopsy to help confirm the diagnosis or to help determine how widespread the lymphoma is. This is more likely to be done if the doctor detects enlarged lymph nodes, either during a physical exam or with imaging tests (see below). Excisional or incisional lymph node biopsy: This is the most common type of lymph node biopsy. In this procedure, a surgeon cuts through the skin to remove either the entire lymph node (excisional biopsy) or a small part of a large tumor (incisional biopsy). If the node is just under the skin, this is often a simple operation that can be done with local anesthesia. But if the node is inside the chest or abdomen, the patient will be asleep or deeply sedated during the biopsy. Removing a lymph node almost always provides enough tissue to diagnose the exact type of lymphoma. This type of biopsy is preferred by most doctors, if it can be done without too much discomfort to the patient. Fine needle aspiration (FNA) biopsy: In an FNA biopsy, the doctor uses a very thin, hollow needle attached to a syringe to withdraw (aspirate) a small amount of tissue from a tumor. If an enlarged node is just under the skin, the doctor can aim the needle while feeling it. If the enlarged node is deep inside the body, the doctor can guide the needle while viewing it with ultrasound or a CT scan (see “Imaging tests” below). An FNA does not require surgery, but in some cases it doesn’t remove enough tissue to make a definite diagnosis of lymphoma. But advances in lab tests (discussed later in this section) and the growing experience of many doctors with FNA have improved the accuracy of this procedure. Some doctors will use FNA in patients already diagnosed with lymphoma of the skin to confirm that an enlarged lymph node also contains lymphoma.

Other Types of Biopsies

These procedures may sometimes be done to confirm a diagnosis of lymphoma, but they are more often done to help stage (determine the extent of) a lymphoma that has already been diagnosed. Not everyone with lymphoma of the skin needs these tests. Bone marrow aspiration and biopsy: These procedures are sometimes done after lymphoma has been diagnosed to help figure out if it has reached the bone marrow. The two tests are often done at the same time. The samples are usually taken from the back of the pelvic (hip) bone, but in some cases they may be taken from other bones. In bone marrow aspiration, you lie on a table (either on your side or on your belly). The doctor cleans the skin over the hip and then numbs the area and the surface of the bone by injecting a local anesthetic. This may cause a brief stinging or burning sensation. A thin, hollow needle is then inserted into the bone and a syringe is used to suck out a small amount of liquid bone marrow. Even with the anesthetic, most patients still have some brief pain when the marrow is removed. A bone marrow biopsy is usually done just after the aspiration. A small piece of bone and marrow is removed with a slightly larger needle that is pushed down into the bone. This may also cause some brief pain. Once the biopsy is done, pressure will be applied to the site to help stop any bleeding. Lumbar puncture (spinal tap): This test looks for lymphoma cells in the cerebrospinal fluid (CSF), which is the liquid that bathes the brain and spinal cord. Most people with skin lymphoma will not need this test. But doctors may order it if a person has symptoms that suggest the lymphoma might have reached the brain. For this test, you may be asked to lie on your side or sit up. The doctor first numbs an area in the lower part of the back over the spine. A small, hollow needle is then placed between the bones of the spine to withdraw some of the fluid.

Treatments

  • When the cancer is localized, radiation therapy is often effective. If it has spread, chemotherapy is the usual treatment.
  • New drugs have recently been developed for the treatment of cutaneous lymphomas, some of which can be given as a pill that is swallowed.