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$Unique_ID{BRK04027} $Pretitle{} $Title{Mycosis Fungoides} $Subject{Mycosis Fungoides Granuloma Fungoides Vidal-Brocq Mycosis Fungoides Discoid Lupus Erythematosus Eczema Leprosy Lichen Planus Lymphocytic Infiltrate of Jessner (Benign Lymphocytic Infiltrate of the Skin) Lymphocytic Leukemia, Chronic Lymphocytoma Cutis Parapsoriasis Lichenoides Chronica (Parapsoriasis Varioliformis Chronica) Sezary Syndrome (Sezary Reticulosis Syndrome; Sezary Erythroderma) } $Volume{} $Log{} Copyright (C) 1988, 1989, 1991 National Organization for Rare Disorders, Inc. 458: Mycosis Fungoides ** IMPORTANT ** It is possible the main title of the article (Mycosis Fungoides) is not the name you expected. Please check the SYNONYMS listing on the next page to find alternate names, disorder subdivisions, and related disorders covered by this article. Synonyms Granuloma Fungoides DISORDER SUBDIVISIONS Vidal-Brocq Mycosis Fungoides Information on the following disorders may be found in the Related Disorders section of this report: Discoid Lupus Erythematosus Eczema Leprosy Lichen Planus Lymphocytic Infiltrate of Jessner (Benign Lymphocytic Infiltrate of the Skin) Lymphocytic Leukemia, Chronic Lymphocytoma Cutis Parapsoriasis Lichenoides Chronica (Parapsoriasis Varioliformis Chronica) Sezary Syndrome (Sezary Reticulosis Syndrome; Sezary Erythroderma) General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Mycosis Fungoides is a chronic progressive lymphocyte disorder arising in the skin. 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 plaques develop which are initially small and elevated. The plaques may enlarge and run together resembling a skin disorder known as exfoliative dermatitis. The lymph nodes may develop another disorder (lipomelanotic reticulosis), characterized by abnormal development of cells called macrophages, and black colored fatty tissue. Additionally, inflammation of the lymph nodes (lymphadenitis) may occur. STAGE III: The third stage is the fungoid or tumor stage. Tumors appear resembling mushrooms which are round or lobulated. Lesions are 1 to 15 cm (1/2 to 6 inches) in diameter, bluish or red-brown in color, with ulcerations. Skin layers may thicken and atypical lymphoid cells may infiltrate the upper skin layer in bands. These cells may also infiltrate the clear spaces in the lower skin layers causing necrosis. STAGE IV: This disorder may next spread throughout the body, marked by fever, weight loss, and anemia. There may be gastrointestinal involvement with or without ulceration of the intestines. The liver and spleen may become enlarged. Coughing and difficulty swallowing (dysphagia) may also occur. 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. It is probably a malignant growth of lymph tissue (lymphoma) originating in the skin, or possibly a young blood cell (reticulum cell) lymphoma. Affected Population Mycosis Fungoides rarely occurs before age 40. It affects males twice as often as females. Related Disorders 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 spots (macules) and plaques displaying redness (erythema), plugging of follicles, scales, vascular lesions (telangiectasia), and wasting (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. The prognosis with treatment is good. (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. 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. 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. Therapies: Standard Mycosis Fungoides may be treated with electron beam radiation, local application of the cytotoxic alkylating drug mechlorethamine and psoralen. Ultraviolet A (PUVA) radiation or exposure to sunlight may also be helpful. Therapies: Investigational In advanced stages of Mycosis Fungoides, injections into the muscles with high-dose recombinant leukocyte A interferon was tested in clinical trials. Initial results indicate this treatment may be effective. Side effects can be reduced by lowering the dosage. However, more research is needed before this treatment can be approved for safe, general use. Cyclosporine (Sandimmune) may be of potential benefit for treating a number of dermatologic diseases. These include Pemphigus and Bullous Pemphigoid, Posterior Uveitis and Behcet's Syndrome, collagen vascular disorders such as severe Dermatomyositis, Sjogren's Syndrome, and Scleroderma, Mycosis Fungoides, and Alopecia Areata. 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 which 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. 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. This disease entry is based upon medical information available through March 1991. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Mycosis Fungoides, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 The Mycosis Fungoides Network Dept. of Dermatology, Pavillion A-3 U C Medical Center Cincinnati, OH 45267-0523 (513) 558-4644 American Cancer Society 1599 Clifton Rd., NE Atlanta, GA 30329 (404) 320-3333 The Skin Cancer Foundation 475 Park Ave. South New York, NY 10016 (212) 725-5176 NIH/National Cancer Institute 9000 Rockville Pike, Bldg. 31, Rm. 1A2A Bethesda, MD 20892 1-800-4-CANCER The National Cancer Institute has developed PDQ (Physician Data Query), a computerized database designed to give doctors quick and easy access to many types of information vital to treating patients with many types of cancer. To gain access to this service, a doctor can contact the Cancer Information Service offices at 1-800-4-CANCER. Information specialists at this toll-free number can answer questions about cancer prevention, diagnosis, and treatment. References ALPHA-INTERFERON 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.