Overview: What is cutaneous lymphoma?
The information in this section has been gathered from existing peer-reviewed and other literature and has been reviewed by expert dermatologists on the CSPA Medical Advisory Board.
Cutaneous lymphoma is a general term for many lymphomas (cancers of the lymph nodes and lymph system) of the skin, including cutaneous T-cell lymphoma, mycosis fungoides, Sézary syndrome, cutaneous anaplastic large-cell lymphoma, adult T-cell leukemia/lymphoma, peripheral T-cell lymphoma, lymphomatoid granulomatosis, granulomatous slack skin disease, B-cell lymphoma, and pagetoid reticulosis, to name a few.
Lymphomas are cancers of the lymphatic system, which consists of a network of vessels, lymph nodes, and a fluid called interstitial fluid. This fluid helps protect our bodies by acting as a defence against infection, and helps feed our body’s tissues by carrying fats and vitamins from our digestive tract to our tissues. The fluid is cleaned via our lymph nodes and is transported into our blood stream via the lymph vessels. The fluid consists mainly of water, protein, molecules, salts, glucose and white blood cells.
Cutaneous T-cell lymphoma (CTCL) is the most common of the skin lymphomas. Mycosis fungoides (MF) is the term for the most prevalent type of CTCL. It is a low-grade lymphoma that primarily affects the skin. Generally it has a slow course and often remains confined to the skin. Over time, there is a low risk (less than 10 per cent) of progression to the lymph nodes and internal organs.
Sézary syndrome (SS) is the leukemic variant of CTCL, meaning it is a cancer that affects the blood cells. Symptoms include widespread redness and scaling of the skin (erythroderma), often with severe itching. Lymph nodes become enlarged, and the malignant T-cells from the skin are also found circulating in the bloodstream.
Scientists are continuing to research cutaneous lymphomas, as no single cause has been found. There is no research indicating that it is hereditary. Studies have also failed to find a connection between chemical exposure, environment, pesticides, radiation, allergies or occupational environments. Exposure to Agent Orange may be a risk factor for developing CTCL-MF in Vietnam War veterans, but no direct cause-effect relationship has been established.
- Cutaneous T-cell lymphoma affects more males than females.
- It is more common after the age of 50.
- CTCL is a rare disease, with approximately one new case per year in a population of 100,000 people.