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Skin lymphoma

This page was reviewed under our medical and editorial policy by

Maurie Markman, MD, President, Medicine & Science at CTCA.

This page was reviewed on June 20, 2022.

Skin lymphoma, also called cutaneous lymphoma, is a less common type of cancer. Unlike other skin cancers, it’s not caused by exposure to the sun.

Skin lymphoma falls under the category of non-Hodgkin lymphomas. In 2022, it’s estimated 80,470 new cases of non-Hodgkin lymphomas will be diagnosed in America, but skin lymphoma makes up only about 4 percent of that total, according to the American Cancer Society.

More skin lymphomas have been diagnosed in recent decades, though researchers aren’t sure why.

What is skin lymphoma?

Lymphoma is a type of slow-growing cancer that begins in the lymphocytes (white blood cells). Lymphoma may also originate in lymphoid tissues, which are found in skin, bone marrow or the spleen and a few other organs.

Hodgkin and non-Hodgkin are the two main types of lymphoma. The term skin lymphoma (or cutaneous lymphoma) is used to describe non-Hodgkin lymphoma that begins only in the skin.

Causes and risk factors of skin lymphoma

The causes of skin lymphoma are not completely clear. However, these risk factors have been linked to the disease:

  • Age: Skin lymphomas are more common in adults in their 50s and 60s, although they may affect people at any age.
  • A weakened immune system: This includes those with a weaker immune system due to disease and those who take medications that weaken the immune system.
  • Infections: Some viruses and bacteria, including Epstein-Barr virus and Borellia (which causes Lyme disease), may cause skin lymphomas.
  • Race: One type of skin lymphoma, called mycosis fungoides, is more common in African Americans.
  • Gender: Skin lymphoma appears to be more common in males.

Skin lymphoma symptoms

Skin lymphomas tend to be highly visible. They may be:

  • Thick (plaques)
  • Flat (patches)
  • Small and pimple-like (papules)
  • Large lumps under the skin

The lesions may feel itchy or scaly. Larger lesions are prone to ulcerating, or breaking open.

Symptoms of skin lymphoma may also show up in other ways. Patients with skin lymphomas sometimes report:

  • Excessive sweating
  • Fatigue
  • Fever
  • Unintended weight loss

Types of skin lymphoma

There are many types of skin lymphomas. They’re classified as T-cell lymphomas or B-cell lymphomas. These classifications are determined by:

  • Where the lymphoma starts (in the T lymphocytes or B lymphocytes)
  • How the lymphoma appears under a microscope
  • If certain types of proteins have been found on lymphoma cells

T-cell skin lymphomas

Mycosis fungoides: One of the most common types of T-cell skin lymphomas is mycosis fungoides, which affects nearly half of skin lymphoma patients. Patchy, scaly, itchy lesions are one common first symptom of mycosis fungoides, and they may be the only symptom for some patients. It’s also possible for this type of skin lymphoma to cause more solid tumors of the skin. Mycosis fungoides may invade the lymph nodes and certain organs, including the liver. Although mycosis fungoides often grow slowly, they may grow more quickly in older adults.

Sezary syndrome: One aggressive type of T-cell skin lymphoma is Sezary syndrome, which many believe to be an advanced form of mycosis fungoides. However, Sezary syndrome is a distinct disease affecting most or all of the skin, causing an itchy, scaly, red rash that may appear as a bad sunburn. Sezary syndrome may spread quickly and is often hard to treat.

Other types of T-cell skin lymphomas include:

  • Adult T-cell leukemia/lymphoma
  • Lymphomatoid papulosis
  • Subcutaneous panniculitis-like T-cell lymphoma

B-cell skin lymphomas

B-cell skin lymphomas are also called cutaneous B-cell lymphomas.

Primary cutaneous follicle center lymphoma: The most common type of B-cell lymphoma is primary cutaneous follicle center lymphoma, which appears as groups of pimples or nodules on the upper body, forehead or scalp. It occurs most often in middle-aged adults. Radiation therapy is often recommended.

Primary cutaneous marginal B-cell lymphoma: Another type is primary cutaneous marginal B-cell lymphoma, which is a treatable, slow-growing lymphoma. It appears as large red or purple pimples, flat lesions or bumps on the arms or upper body.

Primary cutaneous diffuse large B-cell lymphoma: Primary cutaneous diffuse large B-cell lymphoma is a type of lymphoma that often begins on the lower legs. It may spread to the lymph nodes and internal organs.

Diagnosis and testing

A skin lymphoma diagnosis may be challenging because it’s sometimes mistaken for other skin conditions that may have symptoms similar to those known to be linked with cancer. A dermatologist may be the first doctor to examine a potential skin lymphoma and to take a biopsy. A pathologist will study the biopsy for cancer. Because skin lymphoma is less common, any biopsies may need to be sent to larger medical centers that more commonly treat skin lymphoma.

Tests used to diagnose skin lymphoma include:

Skin lymphoma treatments

Skin-directed treatments

Some therapies for cutaneous lymphoma are skin-directed, meaning they focus on the skin. Skin-directed treatments for cutaneous lymphoma include:

  • Surgery, which may be used when only one or a few lesions have been found
  • Radiation therapy, which uses high-energy rays to destroy cancer cells—most commonly electron beam radiation
  • Phototherapy, also called UV (ultraviolet) light therapy
  • Topical therapy, which uses chemotherapy drugs, corticosteroids, retinoids or immunotherapy placed directly on the skin

Whole-body treatments

For fast-growing skin lymphoma, doctors may use systemic, whole-body treatments including those below.

Extracorporeal photopheresis involves obtaining blood and treating it with a light-sensitizing medication and UV light. The blood is then mixed with the rest of the blood and infused into the patient. A patient may receive this type of treatment for two days, then have it repeated every few weeks. It may help destroy some lymphoma cells and improve the body’s immune response against other lymphoma cells.

Chemotherapy uses specific drugs to kill cancer cells. Although not used frequently for skin lymphoma, it may be an option for more advanced cancer that’s not improving with other treatments. Targeted and biologic therapies are drugs that may improve the immune system’s ability to attack lymphoma cells or target certain parts of lymphoma cells.

Targeted and biologic therapies are newer treatments for skin lymphomas and cancer in general.

Retinoids may be an option for mycosis fungoides and Sezary syndrome. Retinoids are related to vitamin A.

Stem cell transplants are used to deliver stronger chemotherapy doses along with blood-forming stem cells that may restore bone marrow. This treatment may be an option if other lymphoma treatments aren’t working. The prognosis for skin lymphoma patients treated with stem cell transplants is still under investigation.

Skin lymphoma survival rate

Survival rates for skin lymphoma vary depending on the type. Many skin lymphomas are diagnosed in the early stages. The most common type of skin lymphoma, mycosis fungoides, is most often slow-growing and caught early. In general, when treatment for skin lymphoma starts in the disease’s early stages, many patients have positive outcomes. Many patients with mycosis fungoides have long life expectancies, though in some cases, the disease may grow more quickly and become more challenging to treat, especially in older people. Several other types of slower-growing B-cell and T-cell skin lymphomas may be treatable if caught early, and most patients may expect a good prognosis. However, more advanced types of skin lymphomas that penetrate the skin and spread to other tissues and lymph nodes may have a less positive prognosis.

For example, about half of patients with primary cutaneous T-cell lymphoma are alive 32 months after treatment (called median survival), according to National Cancer Institute Surveillance, Epidemiology, and End Results (SEER) Program data.

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