Cutaneous Lymphoma Facts

What Is Cutaneous Lymphoma?

Cutaneous lymphoma (also called lymphoma of the skin) is a rare type of non-Hodgkin lymphoma that affects the skin. According to the American Cancer Society, there are over 80,000 cases of non-Hodgkin lymphoma diagnosed each year, only 4% of which are skin lymphomas.

Cutaneous lymphomas result in rashes, and sometimes tumors, which can be mistaken for other skin conditions. In a small number of people the disease may migrate to the lymph nodes or internal organs, causing serious complications. Skin lymphomas are rarer and behave very differently than systemic lymphomas, and usually require different treatments.

There are several subtypes of skin lymphoma that are based on the type of cell in which the disease starts, including:

  • T-cell lymphoma
  • B-cell lymphoma
  • NK-cell lymphoma

Among skin lymphomas, T-cell lymphomas are the most commonly diagnosed.

How Lymphoma Develops

Lymphoma develops when lymphocytes — infection-fighting white blood cells in the immune system — become abnormal and grow and divide uncontrollably into tumors. Lymphocytes are the main part of the immune system and circulate throughout the body responding to bacteria and viruses.

With lymphoma, abnormal white blood cells grow in number (also causing the lymph nodes to swell) not because of an infection, but because of a defect inside the cells. Instead of fighting disease, these cells, which have no real purpose in the body, grow and take up space.

Symptoms Of Cutaneous Lymphoma

The physical symptoms of cutaneous lymphoma vary from patient to patient. They include:

  • Larger papules or lumps: Pimple-like lesions
  • Patches: Flat, scaly lesions
  • Plaques: Thicker, raised lesions
  • Tumors: Raised lumps
  • Erythroderma: A red rash that can cover most of the skin on the body

Other symptoms, such as weight loss, fever, profuse sweating and severe itching may also appear with skin lymphoma. Symptoms of other medical conditions may mirror those of cutaneous lymphoma. If you are treated for those conditions, or if your symptoms last for several weeks despite medical treatment, you may need further consultation to rule out cutaneous lymphoma.

Cutaneous T Cell Lymphomas

There are two main types of lymphocytes: B lymphocytes (B cells) and T lymphocytes (T cells). Lymphomas are classified according to which cell type they are with skin lymphomas divided into either B or T cell lymphomas. Cutaneous T-cell lymphomas make up 75 to 80 percent of cutaneous lymphomas. Most CTCLs are indolent (slow growing) and not life threatening.

The two most common CTCLs are:

  • Mycosis Fungoides: Mycosis fungoides (MF) makes up about half of all cutaneous lymphoma cases. It tends to show up as flat, scaly, red lesions called patches; thicker, raised lesions called plaques; and raised lumps called tumors. In the early stages, MF can be easily confused with other skin problems, such as eczema, psoriasis, or dermatitis, making it difficult to diagnose.
  • Sezary Syndrome: With this condition, patients develop a red rash that covers almost the entire body (called erythroderma). Unlike other types of CTCLs, which affect only the skin, Sezary syndrome affects both the skin and the blood. Lymphoma cells called Sezary cells are found in the skin and the blood, and lymph nodes are often enlarged. Sezary syndrome tends to grow and spread faster than mycosis fungoides. It is also more difficult to treat.
Other types of CTCL include:
  • Lymphoproliferative Disorders: This group includes Primary Cutaneous CD30-Positive disease and its two main subtypes, lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large cell lymphoma (C-ALCL); along with Lymphomatoid Papulosis and Primary Cutaneous Anaplastic Large Cell Lymphoma.
  • Subcutaneous Panniculitis-Like T Cell Lymphoma
  • Primary Cutaneous Peripheral T Cell Lymphoma, Unspecified
  • Primary Cutaneous Aggressive Epidermotropic CD8+ Cytotoxic T Cell Lymphoma
  • Primary Cutaneous Gamma/Delta T-Cell Lymphoma
  • Primary Cutaneous CD4+ Small/Medium Sized Pleomorphic T Cell Lymphoma

Cutaneous B Cell Lymphomas

Cutaneous B cell lymphomas (CBCLs) are much rarer than cutaneous T-cell lymphomas and make up about 20 to 25 percent of cutaneous lymphomas. More common systemic B cell lymphomas, such as Hodgkin’s disease, can spread to the skin and mimic a CBCL.
The three main types of CBCL are:
  • Primary Cutaneous Marginal Zone B Cell Lymphoma
  • Primary Cutaneous Follicle-Center Lymphoma
  • Primary Cutaneous Diffuse Large B Cell Lymphoma, Leg Type
More detailed information about skin lymphoma may be found at the American Cancer Society and Leukemia and Lymphoma Society websites.

What Increases Risk Of Cutaneous Lymphoma?

The exact causes of cutaneous lymphoma are not known, although some risk factors are associated with developing the disease, including being older than 50 and being male. And cutaneous lymphoma is more often diagnosed in African Americans than other groups. While risk factors may be useful in identifying high-risk individuals, they do not determine whether a person develops a disease.

So far, large studies have not linked the environment, chemicals, or infections to developing cutaneous lymphoma.

Skin Cancer Prevention

Sun Safety Tips from City of Hope Cancer Center
Download the sun safety guide

Ultraviolet damage is cumulative, meaning it begins building up in your childhood, so prevention should start at a young age. The best ways to lower your risk for skin cancer include shielding the skin as much as possible from UV radiation:

  • Use sunscreen and reapply at least every two hours
  • Wear protective clothing, including fabrics not easily penetrated by UV light
  • Wear other protective items such as hats and sunglasses
  • Stay in the shade during peak hours when the sun’s UV rays are most intense (from 10 a.m. to 4 p.m.)
  • Avoid tanning salons