Cutaneous B-cell lymphoma (CBCL) is a rare type of non-Hodgkin lymphoma that develops in the B lymphocytes (or B cells) and affects the skin — hence the term “cutaneous.” Skin lymphomas usually develop in the skin without affecting any other areas of the body at the time of diagnosis.

Compared with other types of skin cancers, cutaneous lymphomas are very rare. To gain a better understanding of skin lymphomas, MyLymphomaTeam spoke to Dr. Kimberly Bohjanen, a professor of dermatology at the University of Minnesota Medical School. “While we think of more common skin cancers, like basal cell carcinoma or melanoma, with an incidence rate per 100,000 people, we talk about the cutaneous lymphomas with an incidence rate of per million people,” Dr. Bohjanen explained.

According to the Cutaneous Lymphoma Foundation, the most common type of cutaneous lymphoma is cutaneous T-cell lymphoma, which develops from T lymphocytes. In contrast, cutaneous B-cell lymphoma makes up only 20 percent to 25 percent of cutaneous lymphomas. Despite its rarity, more than 100 members of MyLymphomaTeam report having cutaneous B-cell lymphoma.

Symptoms of Cutaneous B-Cell Lymphoma

The primary symptom of cutaneous B-cell lymphoma is the formation of a lump or multiple lumps in the skin. These lumps, called papules, often appear as small, solid, raised areas of skin. They may be the same color as your skin or may develop into larger red or purple lumps called tumors or nodules. Lesions may also develop into thicker — but still relatively flat — areas of skin called plaques.

Cutaneous B-cell lymphoma (DermNet NZ)

CBCL skin lesions like papules or nodules most commonly form on the neck, head, back, or legs. You may have just one papule or plaque or several. Lesions may be spread widely apart or grouped together. In some cases, papules break down, or ulcerate, and become infected.

As Dr. Bohjanen noted, the symptoms of CBCL may not always be obvious based on appearance alone. Cutaneous lymphoma does not look the same as many other skin cancers. “It would be very hard for the average person to pick out that they had a cutaneous lymphoma,” she explained. “It’s not usually a bleeding spot or a changing mole.”

Furthermore, there can be wide variations in the symptoms of CBCL. Dr. Bohjanen explained that the biggest sign of CBCL is often a persistent lesion, such as a nodule or bump, that doesn’t respond to standard treatments like cortisone creams.

Subtypes of Cutaneous B-cell Lymphoma

There are four types of cutaneous B-cell lymphoma. They are differentiated by where they develop and the characteristics of the B cells from which they arise. Which subtype of CBCL a person has will be determined during the process of diagnosis.

Low-grade, or slow-growing, CBCL subtypes are more common and respond well to milder treatment options. These subtypes include:

• Primary cutaneous follicle center lymphoma
• Primary cutaneous marginal zone B-cell lymphoma

High-grade, or fast-growing, subtypes of CBCL include:

• Primary cutaneous diffuse large B-cell lymphoma, leg type
• Intravascular diffuse large B-cell lymphoma

Some types of lymphoma may begin inside the body and spread to the skin, but these are not cutaneous (skin) lymphomas. A skin lymphoma, like CBCL, does not exist in any other part of the body at the time it is diagnosed.

Diagnosis of Cutaneous B-Cell Lymphoma

Diagnosing cutaneous B-cell lymphoma can take months or years from the time the nodules or rash first appears. CBCL can mimic the appearance of many other skin conditions. A skin biopsy — the removal and testing of a small sample of tissue — is usually required to diagnose CBCL.

Cutaneous lymphomas are, for the most part, a very slow-growing or indolent type of lymphoma. Changes in the skin may not be noticeable for many years, despite the presence of abnormal white blood cells. Because of this, it may take several biopsies over the course of many years before a diagnosis of cutaneous B-cell lymphoma is confirmed.

Skin biopsies can be studied to determine the difference between T-cell and B-cell lymphomas. After collecting the tissue sample, a pathologist will prepare it with stains and examine it under a microscope.

Other tests and procedures used to diagnose cutaneous B-cell lymphoma include:

• A physical exam — Your doctor will examine your skin, check for swollen lymph nodes, and ask about other symptoms.
• Blood tests — Blood tests for lymphoma involve analyzing a sample of your blood for the presence of lymphoma cells.
• Imaging tests — Your doctor may use images from computerized tomography (CT) and positron emission tomography (PET) scans to help diagnose lymphoma.
• Bone marrow biopsy — A sample of your bone marrow is collected with a needle and tested for the presence of lymphoma cells.

Conditions Commonly Mistaken for Cutaneous B-Cell Lymphoma

Cutaneous lymphomas may be difficult to diagnose because they often resemble other, more common skin conditions. Cutaneous B-cell lymphoma may be mistaken for:

• Autoimmune conditions, such as psoriasis and lupus
• Allergic reactions to metals, cosmetics, medications, or sunlight
• Fungal skin infections, such as ringworm (Tinea corporis)
• Lymphocytoma cutis (also known as pseudolymphoma), a skin condition triggered by infections such as shingles, as well as certain drugs, tattoo dyes, vaccinations, insect bites, and scabies

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

Staging Cutaneous B-Cell Lymphoma

There are multiple stages of CBCL, each describing how advanced the lymphoma is and which parts of the body are affected. Staging is determined during the diagnostic process for CBCL.

“Staging is a very important part [of diagnosis] because it tells doctors where you are with the lymphoma,” explained Dr. Bohjanen. “Often, that will help us to determine what treatments you need.” Staging is vital, as the types and stages of lymphoma respond differently to various combinations of treatments.

There are four main stages of lymphoma, numbered 1 to 4, which generally describe how many lymph nodes and places in the body are affected. However, cutaneous lymphomas are staged differently, based on the area of the involved skin. A staging for cutaneous B-cell lymphoma does not have a direct bearing on a person’s prognosis.

If your doctor determines your lymphoma is only on the skin and nowhere else in the body, the stage will likely be Stage 1E. The “E” indicates the lymphoma is extranodal, meaning it has developed outside the lymph nodes.

The letters “A” or “B” may also follow your stage of lymphoma, to indicate whether you are experiencing the systemic lymphoma reactions known as “B symptoms.” The letter “A” means you do not have any of the following B symptoms:

• Severe, drenching night sweats
• Unintentional weight loss
• Unexplained fevers above 100.4 F

Cutaneous lymphoma is also staged with the letters “T,” “N,” and “M.” The letter “T” with a number between 1 and 3 indicates the number and size of tumors or nodules. The letter “N” and a number between 0 and 3 indicates how many lymph nodes are affected (if any). Lastly, the letter “M” and a 0 or 1 describes whether the lymphoma has metastasized (spread to other parts of the body beyond the skin).

Treatment for Cutaneous B-Cell Lymphoma

For some people with indolent types of CBCL who are experiencing no symptoms or disease progression, a doctor may recommend a watchful waiting period. During this time, you will be monitored at regular intervals until a doctor deems active treatment necessary.

Treatment options for cutaneous B-cell lymphoma can include topical steroids, surgery, chemotherapy, or radiation therapy. For people who have just one or a few tumors or nodules, the best treatment may be surgical removal followed by regular monitoring for any return symptoms. About 50 percent of people with CBCL experience a recurrence of tumors after surgery.

For more aggressive or advanced cutaneous B-cell lymphoma, chemotherapy treatments can be either injected into the tumor site or given through an IV. Common chemotherapy treatments prescribed for CBCL include Rituxan (Rituximab) and the chemotherapy combinations CVP or CHOP. CVP includes Cytoxan (Cyclophosphamide), Oncovin (Vincristine), and Prednisone. CHOP is CVP plus Adriamycin (Doxorubicin).

While these treatments are often effective, tumors also return for many people. It is important to continue to have regular follow-up visits with your doctor after completing treatment.

Clinical trials to test the safety and effectiveness of newly developed interventions for CBCL are ongoing and may provide access to experimental treatments for those living with cutaneous B-cell lymphoma.

Side Effects From Treatment for Cutaneous B-Cell Lymphoma

In addition to the symptoms of CBCL, you may experience side effects from more intensive treatments like chemotherapy or radiation therapy. Side effects include:

• Hair loss
• Nausea and vomiting
• Fatigue
• Cognitive changes (sometimes called “brain fog” or “chemo brain”)
• Peripheral neuropathy (pins-and-needles sensations or numbness in the extremities)
• Anemia (low red blood cell count)

These side effects can make daily life more challenging and make treatment more stressful. However, it’s possible to manage them. As one member of MyLymphomaTeam noted, “Each time I start a new procedure or quantity of medication, I get a bit nervous wondering what the side effects might be. Once I get past the initial phase, I develop a routine of self-care and gain control. This makes me feel lots better.”

The symptoms of lymphoma and the side effects caused by treatment can vary widely from person to person. Always report new or worsening symptoms or side effects to your doctor. Luckily, many symptoms and side effects can be managed with medications or lifestyle changes.

Cutaneous B-Cell Lymphoma Prognosis

The prognosis for individuals diagnosed with cutaneous B-cell lymphoma is generally positive. The five-year overall survival rate varies depending on the subtype of CBCL, but for most types it is greater than 90 percent. However, for primary cutaneous diffuse large B-cell lymphoma, leg type, the five-year survival rate is closer to 50 percent. A correct CBCL diagnosis is key to receiving the best treatment for your type.

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Have you been diagnosed with CBCL? How long did it take to get a diagnosis? What treatments have been helpful for you? Comment below or post on MyLymphomaTeam.