Cutaneous T-cell lymphomas (CTCLs) are a rare group of diseases that primarily affect the skin.
They are a cancer of T-cell lymphocytes, a type of white blood cell. Skin lymphoma is very different from systemic lymphoma, and patients can be misled by reading information that confuses the two. Cutaneous T-cell lymphomas can be very varied in appearance on the skin and in their disease course. Fewer than 1 in 100 000 people are diagnosed with CTCL each year.
The most common type of CTCL is mycosis fungoides (MF), which is often not life threatening in early stages, and a more aggressive form called Sézary syndrome (SS). Mycosis fungoides and SS are chronic conditions for which there are many effective treatments. Cutaneous T-cell lymphomas are neither contagious nor inherited. Patients with early-stage, limited skin involvement usually have a good prognosis and do well when treated.
Patients with MF commonly present with a scaly red rash. Many of these patients will have mild involvement in the skin (“patches” or “thin plaques”) with an excellent outcome and low likelihood of the skin condition worsening or developing any systemic symptoms. Other patients with widespread skin involvement (“erythroderma” or extensive “thick plaques”) or with large, thick bumps (“tumor lesions”) are at higher risk of spread to the blood or lymph nodes. Itching is a common symptom in both MF and SS and may be frustrating for many patients.
A diagnosis of CTCL is made by a doctor through skin biopsies, physical examination, and blood tests. Multiple biopsies are usually needed. Cutaneous T-cell lymphomas may look like other skin conditions, so it is important to discuss with your doctor the possibility of this diagnosis—especially if you have not been improving with commonly used treatments for a skin rash. You may also receive additional tests, such as special types of blood tests to look for cancer cells, and radiology imaging studies.
There are many ways to treat CTCL. Your doctor can tailor treatments depending on the type and extent of your disease. Treatments may include only the skin in limited disease (topical, light treatment, or irradiation) or systemic agents in advanced or difficult-to-manage disease (oral, injection, or infusion). It is important to address symptoms associated with CTCL, such as itching, because treating these symptoms may improve quality of life. Because CTCL is so rare, your doctor may refer you to a medical center specializing in the diagnosis and treatment of CTCL.
National Comprehensive Cancer Networkhttps://www.nccn.org/patients/guidelines/nhl-mycosis/files/assets/common/downloads/files/mycosis.pdf
Cutaneous Lymphoma Foundationhttps://www.clfoundation.org/
Conflict of Interest Disclosures: None reported.
Julia Dai, Timothy H. Almazan, Youn H. Kim. Cutaneous T-Cell Lymphomas. JAMA Dermatol. 2017;153(6):620. doi:10.1001/jamadermatol.2017.0361