Mycosis fungoides

Sezary Syndrome

What is Mycosis fungoides?

Mycosis fungoides is a type of lymphoma or cancer of the immune system characterized by the development of red, itchy skin lesions that progress to involve the lymph nodes. The uncontrolled growth of white blood cells called T lymphocytes causes T cell lymphomas, including mycosis fungoides, to appear in the skin. Sezary Syndrome is an advanced form of mycosis fungoides characterized by severe generalized itching and redness (erythema) of the skin.

Mycosis fungoides develops slowly over the course of several years. Many individuals with mycosis fungoides have a long history of other skin disorders. The cause of mycosis fungoides is unknown although it may be a result of viral infection. There are four stages of the disease. Stage 1A is characterized by bright red or brown raised, scaly areas on less than ten percent of the skin surface. These lesions itch and have a tendency to form open sores. Stage 1B involves similar lesions that may vary in color from bluish-red to brown and affect more than ten percent of the skin surface. In stage 2A, lymph node involvement may be present in addition to the skin lesions. In stage 2B, skin tumors (cutaneous tumors) form deep sores. Stage 3 is characterized by swollen lymph nodes in addition to redness over most of the skin along with peeling, scaling, and intense itching. In the final stage, stage 4, the lymph nodes begin to contain cancer cells, indicating that the disease may have spread to the lungs, liver, or other organs. Red skin and large numbers of tumor cells found in the blood indicate the advanced form of the disease known as Sezary Syndrome.

How is it diagnosed?

History: Itching is the most common presenting symptom. The history should include details of previous skin disorders, how long lesions have been present, whether lesions have undergone any change, if lesions are associated with itching or breaks in the skin surface, if they worsen with changes in temperature, or if they are helped by moisturizers.

Physical exam: Depending upon the stage of the disease at initial presentation, the physical examination may reveal areas of redness, ulcerations, enlarged lymph nodes, or enlarged liver or spleen. For accurate staging, the physical examination should indicate the number and type (patch, plaque, tumor, erythroderma) of lesions and their distribution, the percent of skin involved, the number and sites of enlarged lymph nodes, and whether the liver or spleen is enlarged.

Tests: Diagnosis of mycosis fungoides is extremely difficult, often requiring skin biopsies and immunohistochemical staining over a number of years before diagnosis can accurately be made. Chest x-ray, CT scan, and MRI may be used to determine if the disease has spread to internal organs, as can occur in the late stages of the disease. In Sezary Syndrome, the blood smear will show the characteristic malignant Sezary lymphocytes.

How is Mycosis fungoides treated?

Treatment depends upon the stage of the disease. Cortisone ointments may be prescribed to alleviate some of the symptoms of mild mycosis fungoides. Radiation therapy may be used locally or to treat the entire skin (total skin electron beam radiation -TSEB). Cancer-fighting drugs (chemotherapy) may be applied directly to the skin (topical chemotherapy), given orally or by injection.

Photochemotherapy (chemotherapy plus ultraviolet radiation therapy) may also be used. This requires two to three office visits per week of ten to fifteen minutes each. Additionally, immunotherapy may be used in an attempt to boost the individual's own immune system, allowing it to better fight the cancer.

Medications

Information Brand Generic Label Rating
Prednisone Deltasone Prednisone

What might complicate it?

Complications of the disease are related to the stage of the disease and may include ulceration, infection, and bleeding. Chemotherapy or other treatments may cause complications, as well.

Predicted outcome

The prognosis is dependent upon the type and extent of disease at initial presentation. Individuals with early stage disease have a complete response rate (cure rate) of 70 to 90%. Treatment may bring about a remission (disappearance of symptoms) that lasts for many years. Though it can cause death, most individuals with this condition live a normal life expectancy. Individuals with Sezary syndrome are rarely cured and usually die of infection or the effect of progressive lymphoma.

Alternatives

Differential diagnoses include Hodgkin's disease and other chronic skin disorders.

Appropriate specialists

Dermatologist, dermatopathologist, medical oncologist, and radiation oncologist.

Last updated 6 October 2011


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