Cutaneous T-cell lymphoma (CTCL) is a rare type of non-Hodgkin lymphoma that originates in the T lymphocytes (T cells) and spreads to the skin over time. Lymphatic cancers, known as lymphomas, can develop in either T cells or B cells. Healthy T cells are an important part of the immune system, keeping the body safe by destroying cells which have been taken over by pathogens or become cancerous.

Skin lymphomas usually develop in the skin without affecting any other areas of the body at the time of diagnosis. However, CTCL can also affect the lymph nodes, peripheral blood, and internal organs.

To better understand cutaneous T-cell lymphoma, MyLymphomaTeam spoke to Dr. Kimberly Bohjanen, a professor of dermatology at the University of Minnesota Medical School and a specialist in skin lymphomas.

Who Gets Cutaneous T-Cell Lymphoma?

According to Dr. Bohjanen, cutaneous lymphoma is 10 times rarer than other cancers of the skin.

“When you think about the more common skin cancers, like basal cell carcinoma and melanoma, you talk about an incidence rate per 100,000 people,” Dr. Bohjanen explained. “When you talk about the cutaneous lymphomas, you’re talking about an incidence rate per million people.” This is the reason why the condition isn’t more widely discussed.

Recent estimates by the American Cancer Society place newly diagnosed cases of non-Hodgkin lymphoma at 77,240 in the U.S. this year, with only 4 percent being lymphomas of the skin. In the UK, research shows that only about 7 in every 1 million people per year develop any kind of skin lymphoma. Approximately 7 out of 10 cases of skin lymphoma are cutaneous T-cell lymphomas. Cutaneous B-cell lymphoma is even rarer.

Certain factors may increase your likelihood of developing lymphoma. Men are affected by T-cell lymphomas at higher rates than women. T-cell lymphoma is also more likely to affect adults over 60 years of age.

Despite the rarity of cases worldwide, nearly 100 members of the MyLymphomaTeam community have reported that they live with cutaneous T-cell lymphoma.

Symptoms of Cutaneous T-Cell Lymphoma

Dr. Bohjanen explained that the symptoms of cutaneous lymphoma are subtle. In contrast to the more identifiable signs of common skin cancer, such as bleeding spots or moles that change color, she said skin lymphoma causes “more of a rash that just doesn’t go away.” The symptoms persist even with the use of standard topical treatments for rashes.

The symptoms of CTCL largely depend on the stage of cancer a person has. Cutaneous T-cell lymphoma symptoms may sometimes mimic those of other skin conditions, such as eczema, psoriasis, and chronic dermatitis.

Stages of CTCL

Johns Hopkins Medicine describes the stages of CTCL and their symptoms as follows:

• Stage 1 — Lymphoma cells migrate to the upper layers of the skin, causing dry, patchy, scaly, red lesions (or thickened plaques of skin) that mimic eczema. They may also present as bumps on the skin.
• Stage 2 — The red rash and bumps persist and cover up to 80 percent of the skin’s surface. At least one lesion measures 1 centimeter or wider.
• Stage 3 — Most of the skin appears bumpy and may have several tumors or lesions.
• Stage 4 — Almost all the skin remains dry, red, scaly, or bumpy (a condition known as erythroderma) and may have more tumors. During these advanced stages, the disease has also spread to the lymph nodes or to other internal organs, such as the liver and spleen.

Other symptoms that may indicate the presence of CTCL include:

• Enlarged lymph nodes on the neck, under the chin, in the armpits, and near the groin
• An abnormal loss of hair
• Thickened skin on the palms and soles of the feet
• Intense itching and reddened skin over the entire body

Subtypes of Cutaneous T-Cell Lymphoma

Usually, lymphomas are named after the type of cell in which cancer arises. Cutaneous T-cell lymphoma itself is one of many different types of T-cell lymphoma. The two major subtypes of CTCL are mycosis fungoides and Sézary syndrome.

Mycosis Fungoides

Mycosis fungoides is the most common type of cutaneous T-cell lymphoma. It’s considered slow growing, often going for years or decades before it is diagnosed. Usually, mycosis fungoides does not spread beyond the skin. It may progress to lymph nodes and other internal organs in about 10 percent of the cases.

Mycosis fungoides is more common in men than women and affects Black Americans more frequently than white and Asian Americans. It is also more likely to be diagnosed in people over age 50 than in younger people. One rare subtype of mycosis fungoides known as granulomatous slack skin mainly affects men between 30 and 40. Apart from being difficult to diagnose, mycosis fungoides can also present differently across individuals.

Sézary Syndrome

Like most CTCLs, Sézary syndrome is considered indolent (chronic or slow growing), meaning it's typically not life-threatening. Sézary syndrome is distinguished from mycosis fungoides by the presence of Sézary cells in the blood.

While Sézary syndrome has been reported in people of all ages, the condition is more prevalent in adults aged 50 or older. It’s also a bit more common in men than women. There are no known risk factors for Sézary syndrome.

Rare Subtypes of CTCL

One very rare type of CTCL is subcutaneous panniculitis-like T-cell lymphoma. It primarily affects the fatty tissue beneath the skin and doesn’t involve the lymph nodes. The skin lesions in CTCL mimic lipomas. Studied under a microscope, they resemble panniculitis — an inflammation of the adipose tissue — hence the name “panniculitis-like.”

Other subtypes of CTCL include primary cutaneous anaplastic large cell lymphoma and lymphomatoid papulosis.

Diagnosis of Cutaneous T-Cell Lymphoma

The first step of diagnosing CTCL is understanding your medical history. After asking you about your health history, your doctor will then conduct a physical exam. They will examine you for patchy, scaly, or bumpy skin. They may also check for signs of affected lymph nodes and other organs.

Other procedures used to diagnose CTCL may include blood tests, skin biopsies, and imaging.

• Blood tests can provide insight into your condition. In some cases (such as with Sézary syndrome), cancer cells can be found in the blood.
• For a skin biopsy, a small piece of the skin is cut away for testing in the lab.
• Imaging tests, like computerized tomography (CT) and positron emission tomography (PET) scans, check whether the cancer has spread to other parts of the body.

Conditions Commonly Mistaken for Cutaneous T-Cell Lymphoma

A major challenge in the diagnosis of CTCL is that the symptoms often resemble those of other conditions. Conditions that can look like CTCL include:

• Skin diseases, such as eczema, psoriasis, lupus, lichen planus, and chronic superficial dermatitis
• Fungal skin infections, like ringworm
• Reactions to irritants, such as medications, sunlight, and metals in some jewelry
• Pseudolymphoma (lymphoma cutis) triggered by certain medications, vaccinations, insect bites, scabies, tattoo dyes, and infections like shingles

To eliminate these possibilities, the doctor will perform a differential diagnosis. You may have to undergo several tests before your doctor can rule out other conditions and confirm your diagnosis.

Treatment for Cutaneous T-Cell Lymphoma

Treatment options for CTCL depend on the symptoms you exhibit, the stage of your cancer, and your medical history. The treatment approach may also be altered if you stop responding to it. Unfortunately, there is currently no cure for CTCL.

“At this point, we really don’t have any therapies that are considered curative of cutaneous lymphoma,” Dr. Bohjanen said. “We do have many treatments, but a lot of them are aimed at treating the symptoms of the disease.”

Treatment of cutaneous cancer can be skin-directed or systemic — affecting the entire body. Sometimes an oncologist might recommend a combination of both types of treatment. Skin-directed therapy, such as phototherapy (light therapy) and radiation therapy, is usually applied for early-stage lymphomas. Topical chemotherapy, steroids, and retinoids may be administered to limit side effects.

Systemic treatments are usually applied to fast-growing lymphomas. Such therapies include oral chemotherapy, oral retinoids, extracorporeal photopheresis, and targeted and biologic therapies.

Intravenous chemotherapy or radiation may also be applied as a form of treatment. Rarely, if the disease is advanced, a stem cell transplant may be recommended.

Managing Itch in Cutaneous T-Cell Lymphoma

People living with CTCL may experience itching that can range from mildly irritating to intensely uncomfortable.

“Itching is an awful symptom of the disease, and people are miserable with it,” said Dr. Bohjanen. “There are many things we'll try, but unfortunately for a lot of people they still have significant itch. Better itch management is one area that requires more study at this point.”

Doctors may recommend the following treatments for managing itchiness associated with CTCL:

• Moisturizing and over-the-counter remedies
• Topical steroids
• Antihistamines
• Lyrica (Pregabalin) or Neurontin (Gabapentin)
• Antidepressants
• Emend (Aprepitant)
• Phototherapy

To cut down on itching, Dr. Bohjanen recommends a good skin care regimen. Use very mild soaps and keep the skin moisturized. Applying moisturizers right after bathing to seal in moisture can help protect the skin’s defensive barrier.

Cutaneous T-Cell Lymphoma Prognosis

According to the Cutaneous Lymphoma Foundation, most people are diagnosed with CTCL in its early stages and have a life expectancy similar to the general population. For this reason, the prognosis for CTCL is generally good. Some people with CTCL experience long-term remission, and many live without experiencing any symptoms for years.

Advances in research, as well as the progress being made in clinical trials, have helped people with CTCL receive much better care in recent years. Dr. Bohjanen encourages those with CTCL to participate in clinical trials to foster breakthroughs in the field. This, she says, also allows you to take advantage of new developments in the treatment of CTCL.

Getting Support With Cutaneous T-Cell Lymphoma

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Have you or a family member been diagnosed with cutaneous T-cell lymphoma? Do you have any tips for managing itching? Comment below or post on MyLymphomaTeam to begin connecting with others who understand.