Mycosis Fungoides

Mycosis Fungoides

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Mycosis Fungoides is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Granuloma Fungoides

Disorder Subdivisions

  • Vidal-Brocq Mycosis Fungoides

General Discussion

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.

Symptoms

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 "infiltrating stage." 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 d'emblee 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.

Causes

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.

Affected Populations

Mycosis Fungoides rarely occurs before age 40 years. It affects males twice as often as females.

Standard Therapies

A diagnosis of Mycosis Fungoides may be made by a thorough clincial evaluation and a variety of specialized techniques and tests including DNA cytophotometry, nuclear contour analysis, and analysis of T-cell receptor gene rearrangement.



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.



Other treatment for Mycosis Fungoides is symptomatic and supportive.



The orphan drug Methotrexate USP with Laurocapram has received approval for testing from the FDA in the treatment of Mycosis Fungoides. The drug is manufactured by Whitby Research, Inc., Richmond, VA.

Investigational Therapies

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:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

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

Primary Cutaneous Lymphoma - Mycosis Fungoides. T. Nagatani et al.; Gan To Kagaku Ryoho (Jan 1997; 24(1)). Pp. 23-29.



Clinical Stage IA (Limited Patch and Plaque) Mycosis Fungoides. A Long-Term Outcome Analysis. Y.H. Kim et al.; Arch Dermatol (Nov 1996; 132(11)). Pp. 1309-13.



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. T. Horikoshi et al.; Br J Dermatol (Jan 1996; 134(1)). Pp. 130-33.



Combination of Local Puva-Therapy and interferon Alpha-2A in the Treatment of Tumoral Stage Mycosis Fungoides. N. Delpuget-Bertin et al.; Dermatology (1996; 193(1)). Pp. 74-75.



The Head and Neck Manifestations of Mycosis Fungoides. J.A. Brennan; Laryngoscope (May 1995; 105(5 Pt 1)). Pp. 478-80.



Alpha-Terferon Treatment of Cutaneous T Cell Lymphoma and Chronic Lymphocytic Leukemia: K.A. Foon, et al.; Semin Oncol (December 1986: issue 13(4 Suppl 5)). Pp. 35-39.



Combined Total Body Electron Beam Irradiation and Chemotherapy For Mycosis Fungoides: I.M. Braverman, et al.; Journal Am Acad Dermatol (January 1987: issue 16 (1 Pt 1)). Pp. 45-60.



Cutaneous Malignancies and Metastatic Squamous Cell Carcinoma Following Topical Therapies for Mycosis Fungoides: E.A. Abel, et al.; Journal Am Acad Dermatol (June 1986: issue 14(6)). Pp. 1029-1038.

Resources

Skin Cancer Foundation

149 Madison Avenue

Suite 901

New York, NY 10016

Tel: (212)725-5176

Fax: (212)725-5751

Tel: (800)754-6490

Email: info@skincancer.org

Internet: http://www.skincancer.org



American Cancer Society, Inc.

250 Williams NW St

Ste 6000

Atlanta, GA 30303

USA

Tel: (404)320-3333

Tel: (800)227-2345

TDD: (866)228-4327

Internet: http://www.cancer.org



Rare Cancer Alliance

1649 North Pacana Way

Green Valley, AZ 85614

USA

Internet: http://www.rare-cancer.org



Cutaneous Lymphoma Foundation

PO Box 374

Birmingham, MI 48012-0374

USA

Tel: (248)644-9014

Fax: (248)644-9014

Email: info@clfoundation.org

Internet: http://www.clfoundation.org



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



Madisons Foundation

PO Box 241956

Los Angeles, CA 90024

Tel: (310)264-0826

Fax: (310)264-4766

Email: getinfo@madisonsfoundation.org

Internet: http://www.madisonsfoundation.org



Friends of Cancer Research

1800 M Street NW

Suite 1050 South

Washington, DC 22202

Tel: (202)944-6700

Email: info@focr.org

Internet: http://www.focr.org



Cancer Support Community

1050 17th St NW Suite 500

Washington, DC 20036

Tel: (202)659-9709

Fax: (202)974-7999

Tel: (888)793-9355

Internet: http://www.cancersupportcommunity.org/



Lance Armstrong Foundation

2201 E. Sixth Street

Austin, TX 78702

Tel: (512)236-8820

Fax: (512)236-8482

Tel: (877)236-8820

Email: media@livestrong.org

Internet: http://www.livestrong.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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