Cutaneous T-cell lymphoma (CTCL) is a rare idiopathic cancer that tends to occur later in life. It originates in the white blood cell subset of T-lymphocytes that normally help the immune system fight infections. Mutations in the T-cells' DNA cause the cells to develop abnormalities that push them to attack the skin.
"In CTCL, the atypical T-cells have homing devices or markers that cause them to gather in the skin," says Lauren Hughey, MD, a dermatologist at Renew Dermatology in Birmingham.
Hughey, who has been treating cutaneous lymphoma patients for 15 years, explained that by virtue of their training, dermatologists' skin examinations can sometimes give them a window into what's going on inside the body. "I have devoted my career to learning about and treating these cutaneous lymphomas," she says.
There are several subtypes of T-cell lymphomas, each of which can present differently in the skin. Some may have round, itchy, scaly, red patches or areas of skin that appear either lighter or darker in color than the surrounding skin. Others may present as deep, firm plaques under the skin in the fat, or even skin tumors.
"Mycosis fungoides (MF), the most common subtype of CTCL, presents with eczematous patches on the skin. Sezary syndrome, an even more rare blood form of CTCL, causes erythroderma which is skin redness over the entire body," Hughey says.
One of the challenges with cutaneous lymphomas is that it is hard to distinguish from ordinary rashes. "CTCL often appears like eczema or psoriasis, but recognizing the classic distribution of the rash in what we call the sun-protected areas or bathing trunk distribution (hips, buttocks, underarms and breasts) is one of the keys to diagnosing this entity. But even then, it can sometimes take years to get a correct diagnosis," Hughey says. "Once, I had to biopsy a patient 11 times. I knew in my heart that it was CTCL, but I couldn't prove it on biopsy. We kept at it until it finally revealed itself."
Mycosis fungoides typically has a more indolent course, whereas the Sezary syndrome subtype in the blood is much more aggressive. Most stage 1 mycosis fungoides patients, despite its life-long course, will die of something else. "However, a percent of MF patients may progress to have cancer cells in their lymph nodes and in their blood stream," Hughey says. "I always palpate lymph nodes in each patient, and we also do blood work periodically to make sure there are no cancer cells in the bloodstream. A stage 3 or stage 4 patient will need radiation or systemic medications that can include chemotherapies, new targeted drugs or immunotherapies.
"Unfortunately, there is no way to prevent CTCL. We don't know why it occurs, but it can be treated and controlled with therapy. Control is a word that I use a lot with my patients. Unlike other cancers that can be cured with treatment, CTCL is a life-long cancer. I don't like to use words like 'remission' or 'clearance.' Instead, I say 'control.' Our goal is to find a treatment regimen that keeps patients under control and stable with a good quality of life."
Hughey generally sees lower-stage patients in her private practice office, but when a patient's disease becomes more severe, she sees him in a multidisciplinary clinic at UAB that is led by Hughey and oncologist Amit Mehta, MD.
"We see patients in the clinic side-by-side, and it has been extraordinary and a great experience for the patients," Hughey says. "It is like one-stop shopping - patients come for one appointment, and they get both of us in the same room to discuss their case. We each use our expertise from both the dermatology and oncology realms to develop a unique treatment plan for each patient that addresses both skin and systemic issues."
Hughey says there is ongoing research in their field. "Experts are not only studying cutaneous lymphomas to better decipher the pathophysiology of the disease and why it happens. They are also finding out how to treat it," she says.
Hughey points out that science has evolved over the past 20 years, especially in the area of immunotherapies and targeted drugs. "In the past, we had chemotherapy that hit every replicating cell in the body. Now we have new targeted drugs that attach to particular markers to attack just the cancerous T-cells and leave other cells alone," she says. "It is great to see these advancements and the clinical trials at UAB. I am certain that many CTCL patients will benefit in the future from these endeavors."