Article previously published in the Cutaneous Lymphoma Foundation's Forum Spring 2011 newsletter.
For most patients with cutaneous T-cell lymphoma or CTCL, itchiness, also known as pruritus, can range in degree from a minor irritation to a tormenting sensation that can significantly decrease an individual’s quality of life. CTCL-related itching is particularly vexing since it can start as a small fire and progress into a firestorm. An itch can start in a small patch or plaque and, if scratched, will trigger itching in another area, resulting in a full-blown itching frenzy. In a study by Dr. Marie-France Demierre of the Boston University School of Medicine, 88 percent of CTCL patients ranked pruritus among the top causes of distress associated with the condition. (1)
When Treatment is Needed
Pruritus can vary in incidence and severity depending on the type of CTCL. Mycosis fungoides (MF), is the most common type of CTCL, and can appear as flat red patches on the body as well as thicker, raised lesions called plaques. People living with MF typically report various levels of itch, mostly concentrated in plaques, with the most severe levels experienced in advanced cases. The advanced and leukemic form of CTCL is called Sézary syndrome (SS), differentiated by the presence of malignant lymphocytes in the blood and an extensive thin, itchy, reddish colored rash that covers over 80 percent of the body. Sézary syndrome encompasses less than ten percent of CTCL cases, however nearly all afflicted individuals suffer from moderate to severe degrees of pruritus.
Talking to Your Doctor
Above all, talking to your doctor will help determine the best methods of pruritus management. Pruritus is a physiological condition at its root, and appropriate treatment is more likely to be prescribed if the degree of suffering is quantified. When meeting with your doctor, try to describe the severity of itchiness on a scale of one to ten, with one being little to no itch and ten being unbearable and inhibitive to daily functions. In some cases it may be helpful for patients to take a quality of life survey in order for the doctor to better understand how pruritus impacts the individual on an emotional and functional level. This can result in more effective care and increase the level of communication between doctor and patient.
Available Treatment Methods
Although a definitive cause for pruritus has yet to be determined, there are various treatments. A common first-line treatment is antihistamines, which counteract the reactions that occur when histamines are released in the skin, thereby blocking redness, swelling, and itchiness. For most pruritus sufferers, itching becomes noticeably greater just prior to falling asleep, so sedative antihistamines can be particularly effective.
Neurontin (gabapentin), marketed as an anticonvulsant for individuals prone to seizures, has been shown to be effective in treating pruritus. Gabapentin can relieve itching sensations by blocking the effect of specific neurotransmitters and subduing neuronal hyperexcitability.(2) Emend (aprepitant) is another prescription medication used for preventing chemotherapy-induced nausea and vomiting, shown to be effective in reducing pruritus. CTCL patients, particularly those with SS, have notably high levels of the neuropeptide “substance P.” Aprepitant works by blocking certain receptors in the brainstem including a substance P receptor, which is also linked to feelings of nausea. Although the research is fairly new, this drug has shown dramatic itch suppression in several severe cases of SS. (3) There is no generic form of Emend, so insurance coverage may be an issue. Similarly, an antidepressant called Remeron (mirtazamine) has been shown in case studies to relieve pruritus. (4)
Phototherapy is a viable first-line treatment option for patients with MF, especially those patients suffering with pruritus. One of the most effective phototherapy treatments is psoralen plus ultraviolet A (PUVA) which, when utilized in early stage MF, can yield significant improvement including long-lasting disease-free intervals. Narrow-band UVB phototherapy has also proven effective, when administered in thrice weekly sessions of UVB exposure until lesions regress. Common criticisms of phototherapy include a slow response time as well as increasing itch following the first treatments. Additionally, phototherapy has a low response rate for those with SS.
Topical steroids may be effective when used in conjunction with other treatment methods, but tend to be impractical in treating severe cases due to the large surface area. The most effective over-the-counter lotions for less severe cases are those that contain menthol, as its cooling properties can overpower the itching sensation. Much in the same way, applying an ice pack at the onset of an itch may temporarily suppress the discomfort. Be cautious of using analgesics, as they have been shown to aggravate itching episodes. Also, some relief has been reported through alternative methods, such as acupuncture and biofeedback.
To make the most out of whichever treatment method is selected by you and your doctor, good skin care is an important component. There are many simple and inexpensive strategies that can be used to minimize the discomfort caused by pruritus, such as taking a lukewarm shower rather than a steaming hot bath since high temperature water can wash off a layer of hydrating oils that develop naturally on skin. Additionally, washing with fragrance-free soap and applying fragrance-free moisturizing lotion or petroleum jelly, particularly when skin is still moist, can extend hydrating effects and result in reduced itch.
Based on this summary review, it is clear we have more work to do to better understand the root causes of CTCL-related pruritus. Based on the clinical research ongoing with various systemic, phototherapeutic and topical treatments, we are learning more every day. It is also encouraging to know that pruritus has been identified as a main area of focus by the US Cutaneous Lymphoma Consortium. In addition to frequent and specific communication with your medical team, you should visit the Cutaneous Lymphoma Foundation website (www.clfoundation.org) for more information about pruritus and CTCL.
(1) Demierre, M. (2010, September). Mycosis fungoides and Sézary syndrome: the burden of pruritus. Community Oncology, 7(9), 399-404.
(2) The Cutting Edge: Efficacy of Gabapentin in the Management of Pruritus of Unknown Origin. American Medical Association Website. Available at:
http://archderm.ama-assn.org/cgi/reprint/141/12/1507.pdf. Accessed on June 21, 2011.
(3) Booken, N., Heck, M., Nicolay, J. P., Klemke, C. D., Goerdt, S., & Utikal, J. (2011, January 28). Oral aprepitant in the therapy of refractory pruritus in erythrodermic cutaneous T-cell lymphoma. British Journal of Dermatology, 164(3), 665-667.
(4) Clinical Note: Mirtazapine for Pruritus. Journal of Pain and Symptom Management Website. Available at: http://my.clevelandclinic.org/Documents/ Services/Mirtazapine.pdf. Accessed on June 21, 2011.
Dr. John Zic is an Associate Professor of Medicine in the Division of Dermatology at the Vanderbilt University Medical Center in Nashville, Tennessee. Dr. Zic received his undergraduate degree from the University of Notre Dame and medical degree from the Vanderbilt University School of Medicine in 1991. He completed his internship in Medicine at the University of Chicago Hospital and his postgraduate training in Dermatology at the University of Illinois at Chicago Hospital. In 1996 he established the Vanderbilt University Cutaneous Lymphoma Clinic. He is presently the chair of the Education Committee and the Pruritus Task Force of the US Cutaneous Lymphoma Consortium.