Cutaneous B-Cell Lymphoma

What is cutaneous B-cell lymphoma?

Cutaneous B-cell lymphoma (CBCL) is a rare type of cancer that starts in your skin. It’s a form of “extranodal” (outside of your lymph nodes) lymphoma.

Most cases look like a rash or lump(s) on your skin. They usually grow slowly and rarely spread or affect other parts of your body.

CBCL is also called B-cell skin lymphoma.

What are the different types of CBCL?

There are four types of CBCL, depending on how the cells look under a microscope. Each subtype may look different and need distinct treatments.

• Primary cutaneous follicle center lymphoma: This is the most common CBCL subtype. It often appears on your head, neck or torso as a reddish-brown, bumpy rash or nodules. This subtype grows slowly, over months or years.
• Primary cutaneous marginal zone B-cell lymphoma: This slow-growing subtype typically involves pink or red lesions, nodules or tumors. It often develops on your torso or arms.
• Primary cutaneous diffuse large B-cell lymphoma, leg type: This subtype often appears as one or more tumors or nodules on your legs, arms or torso. It’s less common than the other two types and more aggressive, growing over weeks or months.
• Primary cutaneous diffuse large B-cell lymphoma, other: This is a very rare but aggressive subtype. Its appearance varies, but it usually starts on your head, torso and extremities (hands and feet).

Who might get cutaneous B-cell lymphoma?

Anyone can develop CBCL. It’s known to occur in all genders/sexes and age groups, and many racial backgrounds.

How common is cutaneous B-cell lymphoma?

CBCL is rare, affecting only about 4 of every 1 million people.

What causes cutaneous B-cell lymphoma?

Scientists don’t understand what causes CBCL, but it’s not inherited or contagious. They do know that it starts in white blood cells called lymphocytes, part of your immune system.

Lymphocytes can be classified as T cells or B cells. This type of lymphoma involves B cells growing out of control.

What are the symptoms of cutaneous B-cell lymphoma?

CBCL causes a rash, lump, bump, nodule or tumor on your skin (often called lesions). They may look red, purple or brown.

Lesions may be slightly raised and solid but smooth (called papules). They also may be flat, thickened areas of skin (called plaques). Larger bumps are often called nodules or tumors.

The condition may involve only one lesion or several. And lesions may appear on only one part of your body or multiple areas. They may be close together or spread out.

Rarely, some people also experience other symptoms, sometimes referred to as “B” symptoms, including:

• Fever.
• Night sweats.
• Swollen lymph nodes in your neck, armpits or groin.
• Weight loss.

How is cutaneous B-cell lymphoma diagnosed?

CBCL lesions can look like pimples, bug bites, allergic reactions, scabs or skin conditions (such as eczema or psoriasis). Because of this, many people wait to seek medical attention. But if you have any skin abnormalities, you should talk to a healthcare provider, such as your primary care provider or a dermatologist.

Your healthcare provider can diagnose CBCL with a physical examination and skin biopsy. During a physical exam, your healthcare provider will look at your skin all over your body, carefully assessing any abnormalities. During a skin biopsy, they’ll remove a small piece of tissue so an expert can look at it under a microscope. A biopsy can identify skin lymphoma and its subtype.

Your healthcare provider might order staging tests to determine whether the cancer has spread. These tests might include:

• Blood tests.
• Imaging tests such as CT scan or PET scan.
• Bone marrow biopsy.
• Biopsy of the lymph nodes.

What are the stages of B-cell skin lymphoma?

CBCL stage describes whether the cancer has spread and how much. The stage is important because it guides treatment decisions. Your healthcare provider will explain what stage the cancer is in and what it means for you.

Stage 1E (for extranodal) affects only your skin and hasn’t spread anywhere else. It may also be called stage 1AE if you have no additional “B” symptoms, such as weight loss.

If CBCL has spread, healthcare providers use the TNM staging system:

• Tumor: “T” documents the number of papules, nodules or tumors, how big they are and where they are. The numbered scale ranges from less to more serious (1 to 3).
• Nodes: “N” indicates whether lymph nodes are involved, how many and where they are. The scale ranges from less to more serious (0 to 3).
• Metastasis: “M” refers to whether lymphoma has metastasized (spread) beyond your skin or lymph nodes (M1) or not spread (M0).

Can cutaneous B-cell lymphoma be cured?

Most cases of CBCL can be treated and cured, but the condition may come back (recur).

How is cutaneous B-cell lymphoma treated?

Treatment for CBCL depends on the subtype and stage. Treatment options include:

• Active surveillance, or “watch and wait,” which involves monitoring the condition until treatment is necessary.
• Chemotherapy.
• Medications rubbed on your skin (topical) or injected, such as corticosteroids.
• Monoclonal antibodies such as rituximab.
• Radiation therapy.
• Surgical removal.

How can I prevent cutaneous B-cell lymphoma?

Because scientists don’t understand what causes CBCL, there aren’t any proven strategies to prevent it.

What is the survival rate of cutaneous B-cell lymphoma?

Prognosis depends on several factors, especially subtype and stage.

The two slow-growing subtypes have a five-year survival rate of about 95%. That means 95% of people are alive five years after diagnosis. But the more aggressive subtypes have a five-year survival rate of 60%.

Can CBCL come back after treatment?

Skin lymphoma often comes back after treatment. Lesions may appear in the same area or a new area. They’re usually the same subtype as before. But rarely, recurrent CBCL can show up as systemic disease or another type of lymphoma.

So it’s important to perform skin checks often and see a dermatologist or other specialist regularly for check-ups.

How can I take care of myself with CBCL?

If you have CBCL or have had it in the past, it’s important to follow your healthcare provider’s instructions. You’ll need check-ups to make sure the disease doesn’t recur.

The frequency of appointments depends on the type of CBCL and its aggressiveness. You may need a check-up every few weeks, months or annually. Follow-up appointments may involve skin checks, blood work, imaging tests or biopsies.

What should I ask my doctor about cutaneous B-cell lymphoma?

If you’ve been diagnosed with cutaneous B-cell lymphoma, consider asking your healthcare provider:

• What CBCL subtype do I have?
• What stage is it?
• What treatments do you recommend?
• What are the side effects and risks of treatment?
• What’s my prognosis (survival and outcomes)?
• How long will it take before we know whether treatment worked?
• How often should I follow up with you to ensure the lymphoma hasn’t come back?

A note from Cleveland Clinic

Cutaneous B-cell lymphoma is a rare type of cancer of the lymphocytes that starts in your skin. Most cases are slow growing and don’t spread to other areas of your body. But some types can metastasize (spread). If you notice a new bump or rash on your skin, talk to your primary care provider or a dermatologist.