A diagnosis of mycosis fungoides can be very frightening for patients and family members, not least because it is a rare condition and the name is unfamiliar to most people. As with any serious illness, one of the first and most proactive steps that can be taken is education: understand the symptoms and the prognosis, and you will be far better prepared to combat the disease.
What is Mycosis Fungoides?
Also known as Sézary syndrome in its most advanced stages, this disease is a relatively rare type of non-Hodgkins lymphoma. It presents as reddish skin patches and lesions, which are often misdiagnosed as other, more benign skin conditions like eczema and psoriasis. In some cases, skin tumors and plaques (red, thickened areas of skin) may also present as symptoms. The disease primarily affects adults over the age of 50, but non-Hodgkins lymphomas can affect individuals from all age groups.
History of the Disease
First identified by dermatologist Jean-Louis-Marc Alibert in 1806, the name “mycosis fungoides” essentially translates to “mushroom-like fungal disease.” This name is somewhat misleading, as the disease is not related to any type of fungal infection. However, it does describe the tumors that may arise in severe cases, such as the one Alibert first diagnosed. Earlier cases of the disease were most likely thought to be forms of skin infections or allergic reactions, as symptoms can be present within the body for many years before life threatening problems arise.
More About Non-Hodgkins Lymphoma
Non-Hodgkins lymphoma is a cancer of the white blood cells within the body, specifically the lymphocytes that comprise the lymphatic system. The two main types of lymphocytes, B-lymphocytes and T-lymphocytes, respectively combat bacterial and viral infections. B-lymphocytes are most commonly affected, comprising almost 85% of non-Hodgkins lymphoma cases. As with most cancers of the blood, a malignant lymphocyte can infect any part of the body, and may metastasize in the lymph nodes, bone marrow, or any organ.
Cutaneous T-Cell Lymphoma
Mycosis fungoides falls into a rare group of non-Hodgkins lymphomas in which the T-cell lymphocytes within the skin become cancerous. Cutaneous T-cell lymphomas account for only about 10-15% of all non-Hodgkins lymphomas diagnosed in the U.S. each year, though certain cases occur more regularly in Asia and other parts of the world. These types of cancer are often slow moving, and can be present within the skin for many years; it is rare for individuals to be diagnosed before the age of 20, and most cases occur after age 50.
Causes and Risk Factors
There are no known causes for this type of T-cell cutaneous lymphomas. However, the disease is known to occur more frequently in men than in women, and affects people of color and individuals of African descent more often than those with white European ancestry. Some scientists theorize that there are genetic risk factors for the disease, while others posit that chemical and viral exposure may play a role. However, none of these theories have been proven correct; there have been rare cases of familial mycosis fungoides, but no single gene has been identified as the culprit in these instances.
All cancers are potentially life threatening, and should be taken very seriously; in this case, mycosis fungoides is no exception. This disease typically has a mortality rate of 60%, and the sooner it is diagnosed the better. There are a variety of treatment options available; a good doctor will help patients and their families choose the one that will be most effective for their situation.