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รltima actualizaciรณn: October 25, 2013
Aรฑos publicados: 1988, 1989, 1991, 1997, 2003, 2013
NORD gratefully acknowledges Javier Munoz, MD, FACP, Hematology and Oncology, Banner MD Anderson Cancer Center, Gilbert, Arizona, for assistance in the preparation of this report.
Mycosis fungoides is a rare form of T-cell lymphoma of the skin (cutaneous); the disease is typically slowly progressive and chronic. In individuals with mycosis fungoides, the skin becomes infiltrated with plaques and nodules that are composed of lymphocytes. In advanced cases, ulcerated tumors and infiltration of lymph nodes by diseased cells may occur. The disorder may spread to other parts of the body including the gastrointestinal system, liver, spleen, or brain.
STAGE I: The first sign of mycosis fungoides is usually generalized itching (pruritus), and pain in the affected area of the skin. Sleeplessness (insomnia) may also occur. Red (erythematous) patches scattered over the skin of the trunk and the extremities appear. These lesions may resemble other skin disorders such as psoriasis, parapsoriasis, lichen planus, or eczema.
STAGE II: The second stage is called the plaque or ยซinfiltrating stage.ยป Bluish red circular or oval plaques develop on affected areas. The buttocks may be the first area to be affected. Initially, these plaques are typically small and elevated. The plaques may slowly enlarge and run together (coalesce), covering approximately 10 percent of the body. At this point, the lesions may resemble a skin disorder known as exfoliative dermatitis.
Another condition of the lymph nodes may also develop known as lipomelanotic reticulosis. This condition is characterized by abnormal development of certain cells called macrophages and the presence of dark colored fatty tissue. Additionally, inflammation of the lymph nodes (lymphadenitis) may also develop.
STAGE III: The third stage of the disease is the fungoid or tumor stage. Tumors appear that resemble mushrooms; they may appear rounded or lobulated. These ulcerated lesions are typically 1 to 15 cm (1/2 to 6 inches) in diameter and bluish or red-brown in color. Skin layers may become thick and atypical bands of lymphoid cells may infiltrate the upper skin layer. These cells may also infiltrate the clear spaces in the lower skin layers causing skin cell death (necrosis).
In the tumuer dโemblee form of the disease, affected individuals may develop large nodules without previous formation of plaque.
STAGE IV: During this stage, the disorder may spread throughout the body. Symptoms may include a general feeling of ill health (malaise) and weakness, elevated temperatures, weight loss, and anemia. There may be gastrointestinal involvement with or without ulceration of the intestines. The liver and spleen may also become enlarged. In addition, coughing and difficulty swallowing (dysphagia) may occur. In some cases, the heart muscle may also be affected. If the brain is involved, eye pain and loss of clear vision may occur.
The exact cause of mycosis fungoides is not known. Current theories include antigen persistence, retroviruses (e.g., HTLV-1, etc.), and exposure to cancer-causing (carcinogenic) substances.
Mycosis fungoides rarely occurs before age 40 years. It affects males twice as often as females.
A diagnosis of mycosis fungoides may be made by a thorough clinical evaluation and a variety of specialized techniques and tests including DNA cytophotometry, nuclear contour analysis, and analysis of T-cell receptor gene rearrangement.
Treatment
Treatment methods for mycosis fungoides include photochemotherapy (PUVA), topical steroids, short courses of UVB (during winter months), a drug known as topical nitrogen mustard (mechlorethamine), interferons, oral retinoid therapy, and/or photopheresis. In later stages of the disorder, electron beam therapy may prove beneficial.
The FDA has approved mechlorethamine gel for the topical treatment of stage IA-IB mycosis fungoides-type cutaneous T-cell lymphoma. The mechlorethamine gel is applied once per day on the affected skin.
In 2018, Poteligeo (mogamulizumab-kpkc) injection was approved for the treatment of adult patients with relapsed or refractory mycosis fungoides (MF) after at least one prior systemic therapy. This approval provides a new treatment option for patients with MF Poteligeo is manufactured by Kyowa Kirin, Inc.
Other treatment for mycosis fungoides is symptomatic and supportive.
Cyclosporine (Sandimmune) may be of potential benefit for treating a number of dermatologic diseases including mycosis fungoides. Certain types of skin grafts have also shown improvement after cyclosporine treatment, in some cases. However, this drug may also be associated with severe and life-threatening side effects that would limit its use in many patients.
Careful monitoring of this drug by a physician is necessary to guard against possible toxic side effects. Relapses can occur when the drug is discontinued. More research is needed before cyclosporine can be recommended as a treatment for all but the most severe cases of the disorders listed above. Even for the most severe cases its use is still experimental, and long-term effects are unknown.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Toll-free: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]
Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/
For information about clinical trials sponsored by private sources, in the main, contact:
www.centerwatch.com
For more information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
TEXTBOOKS
Textbook of Dermatology, 5th Ed.: R.H. Champion, J.L. Burton, and F.J.G. Ebling, Editors; Blackwell Scientific Publications, 1992. Pp. 2119-28.
JOURNAL ARTICLES
Lessin SR, Duvic M, Guitart J, et al. Topical chemotherapy in cutaneous T-cell lymphoma: positive results of a randomized, controlled, multicenter trial testing the efficacy and safety of a novel mechlorethamine, 0.02%, gel in mycosis fungoides. JAMA Dermatol. 2013;149(1):25-32.
T. Nagatani, et al. Primary Cutaneous Lymphoma-Mycosis Fungoides. Gan To Kagaku Ryoho. 1997;24(1):23-29.
Kim YH, et al. Clinical Stage IA (Limited Patch and Plaque) Mycosis Fungoides. A Long-Term Outcome Analysis. Arch Dermatol.1996;132(11):1309-13.
Horikoshi , et al. A Patient with Plaque-Stage Mycosis Fungoides Has Successfully Been Treated with Long-Term Administration of IFN-Gamma and has been in Complete Remission for more than 6 years. Br J Dermatol. 1996;134(1):130-33.
Delpuget-Bertin N, et al. Combination of Local Puva-Therapy and interferon Alpha-2A in the Treatment of Tumoral Stage Mycosis Fungoides. Dermatology.1996;193(1):74-75.
Brennan JA. The Head and Neck Manifestations of Mycosis Fungoides. Laryngoscope. 1995;105(5 Pt 1):478-80.
Foon KA, et al; Semin Alpha-Terferon Treatment of Cutaneous T Cell Lymphoma and Chronic Lymphocytic Leukemia. SeminOncol. 1986;13(4 Suppl 5):35-39.
Braverman IM, et al. Combined Total Body Electron Beam Irradiation and Chemotherapy For Mycosis Fungoides. Journal Am Acad Dermatol. 1987:16 (1 Pt 1):45-60.
Abel EA, et al. Cutaneous Malignancies and Metastatic Squamous Cell Carcinoma Following Topical Therapies for Mycosis Fungoides. Journal Am Acad Dermatol. 1986:14(6):1029-1038.
NORD y la Fundaciรณn MedicAlert se han asociado en un nuevo programa para brindar protecciรณn a pacientes con enfermedades raras en situaciones de emergencia.
Aprende mรกs https://rarediseases.org/patient-assistance-programs/medicalert-assistance-program/Asegurarse de que los pacientes y los cuidadores estรฉn equipados con las herramientas que necesitan para vivir su mejor vida mientras manejan su condiciรณn rara es una parte vital de la misiรณn de NORD.
Aprende mรกs https://rarediseases.org/patient-assistance-programs/rare-disease-educational-support/Este programa de asistencia, primero en su tipo, estรก diseรฑado para los cuidadores de un niรฑo o adulto diagnosticado con un trastorno raro.
Aprende mรกs https://rarediseases.org/patient-assistance-programs/caregiver-respite/The information provided on this page is for informational purposes only. The National Organization for Rare Disorders (NORD) does not endorse the information presented. The content has been gathered in partnership with the MONDO Disease Ontology. Please consult with a healthcare professional for medical advice and treatment.
The Genetic and Rare Diseases Information Center (GARD) has information and resources for patients, caregivers, and families that may be helpful before and after diagnosis of this condition. GARD is a program of the National Center for Advancing Translational Sciences (NCATS), part of the National Institutes of Health (NIH).
View reportOrphanet has a summary about this condition that may include information on the diagnosis, care, and treatment as well as other resources. Some of the information and resources are available in languages other than English. The summary may include medical terms, so we encourage you to share and discuss this information with your doctor. Orphanet is the French National Institute for Health and Medical Research and the Health Programme of the European Union.
View reportOnline Mendelian Inheritance In Man (OMIM) has a summary of published research about this condition and includes references from the medical literature. The summary contains medical and scientific terms, so we encourage you to share and discuss this information with your doctor. OMIM is authored and edited at the McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine.
View report