Mycosis Fungoides

Disease Overview

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.

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Synonyms

• Granuloma Fungoides

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Subdivisions

• Vidal-Brocq Mycosis Fungoides

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Signs & 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.

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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.

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Affected populations

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

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Disorders with Similar Symptoms

Symptoms of the following skin disorders may resemble those of mycosis fungoides. Comparisons may be useful for a differential diagnosis:

Discoid lupus erythematosus is a chronic and recurrent autoimmune disorder primarily affecting the skin. It is characterized by sharply circumscribed red spots (erythematous macules) and plaques, plugging of follicles, scales, vascular lesions (telangiectasia), and loss of skin tissue (atrophy). There are two varieties: one with lesions above the chin, the other with or without facial involvement but causing skin lesions on the rest of the body. (For more information on this disorder, choose “lupus” as your search term in the Rare Disease Database.)

Eczema (dermatitis) is a common superficial inflammation of the skin, characterized by extremely dry and cracked skin with blisters (when acute), redness, swelling, oozing, crusting, and scaling. It is usually itchy and commonly associated with allergies.

Leprosy (Hansen’s disease) is a chronic infectious disorder caused by bacteria (Mycobacterium leprae). It tends to occur in tropical and subtropical areas of the world. Skin, mucous membranes, eyes and peripheral nerves may be involved. Nerve damage can result in loss of sensation and movement in the face, hands and feet. This in turn can lead to crippling and disfigurement. Blindness may result from eye complications. (For more information on this disorder, choose “leprosy” as your search term in the Rare Disease Database.)

Lichen planus is a recurrent, itchy, inflammatory eruption of the skin which is characterized by small separate, angular spots that may flow together into rough scaly patches. It is often accompanied by lesions in the mouth. Women are most commonly affected by the disorder. (For more information on this disorder, choose “lichen planus” as your search term in the Rare Disease Database.)

Lymphocytic infiltrate of Jessner (benign lymphocytic infiltrate of the skin) is a skin disorder characterized by benign accumulations of lymph cells in the skin, whereas Mycosis fungoides is a malignant infiltration of lymph cells. These small lesions are solid, pink or red, and appear on itchy and reddened areas of the face, neck and/or back. Lesions may remain unchanged and then spontaneously resolve after several years, leaving no scars. (For more information on this disorder, choose “lymphocytic infiltrate of Jessner” as your search term in the Rare Disease Database.)

Chronic lymphocytic leukemia is characterized by an abnormal accumulation of lymph cells from the lymph nodes and tissues. These cells infiltrate the bone marrow and replace the normal blood forming elements. The disorder, almost three times more common in males than in females, occurs chiefly between the ages of 50 to 70, but may occur at any age. (For more information on this disorder, choose “chronic lymphocytic leukemia” as your search term in the Rare Disease Database.)

Parapsoriasis lichenoides chronica (parapsoriasis varioliformis chronica) is a relatively benign, chronic, scaly skin disorder characterized by elevated spots (papules). It may occur at any age and is not easily treatable. (For more information, choose “psoriasis” as your search term in the Rare Disease Database.)

Sezary syndrome (Sezary reticulosis syndrome; Sezary erythroderma) is a generalized redness of the skin (erythroderma) in which areas of the skin fall off in scales. It is caused by infiltration of the skin by young blood (reticular) cells. The disorder is associated with intense itching, loss of hair, swelling, and overdevelopment of the horn layer of the skin (hyperkeratosis). Changes in skin pigment, fingernails, and toe nails may occur. Bone marrow and lymph nodes are normal in patients with this disorder but abnormal young red blood cells may often be found.

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Diagnosis

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.

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Standard Therapies

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.

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Clinical Trials and Studies

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: prpl@cc.nih.gov

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/

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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

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.

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Programs & Resources

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