What is Mycosis Fungoides?
Mycosis fungoides is a malignancy of the skin and is regarded to be the most common form of cutaneous T-cell lymphoma. The term mycosis fungoides is a misnomer for a fungal disease as it is not an infection from fungi but rather a type of non-Hodgkin lymphoma.
Mycosis fungoides is also known as Alibert-Bazin syndrome and belongs to a group of rare cancer that involves the skin. It affects men more than women and usually occurs in individuals over the age of 20 years, although it may also occur in children and adolescents. The disease was first described by a French dermatologist by the name of Jean Louis Marc Alibert during the year 1806 when he recognized the disease in severe case. The skin tumor in severe case was described by Alibert as similar to the mushroom.
Staging cancer is the process in identifying the extent of cancer which plays a vital role in planning the treatment for the patient. The process of staging is made possible through a series of diagnostic tests which help in identifying the spread of the malignant tumor in the body if it has managed to spread to distant parts of the body from the origin of the cancer. Metastasis is the term used to describe the spread of cancer cell after it has broken away from the primary tumor and travel through the lymph nodes or blood.
The staging of Mycosis fungoides is based on the four factors which are then grouped to properly stage the cancer. The four factors considered in staging the Mycosis fungoides include the following:
T stands for the tumor and is categorized according to the extent or amount of skin affection. The tumor or the lymphoma is categorized from T1 to T4.
N in the staging is the factor which describes the extent of the lymphoma in relation to the involvement of the lymph nodes. The node involvement is categorized from N0 to N3 including NX.
M describes the spread or metastasis of the primary tumor to distant parts or organs of the body. The metastasis is categorized from M0 to M1.
B in the staging process of Mycosis fungoides describes the infiltration of the lymphoma in the blood. The involvement of blood is categorized from B0 to B1.
When all the factors and categories have been grouped together, the overall stage of Mycosis fungoides is then determined.
Stage 1 Mycosis fungoides is further divided into stage 1A and 1B. Stage 1A is described as Mycosis fungoides with the presence of lesions but with the absence of tumor. The skin lesion covers less than 10% of the skin surface while the lymph nodes are not noted for enlargement. The spread of the cancer cells to other parts or organs of the body while the involvement of blood may not be sufficient enough. Stage 1B is described with lesions that cover the skin surface for about 10% or more while the lymph nodes are still not enlarged and no spread to other organs or parts of the body can be noted.
Stage 2 Mycosis fungoides is also divided into stage 2A and stage 2B. Stage 2A of Mycosis fungoides is characterized by skin lesions covering more than 10% or at least 80% of the skin surface. The lymph nodes on the other hand are enlarged although the cell patterns are not unusual when seen under the microscope. No spread of lymphoma to other parts or organs of the body can be noted. Stage 2B is described by the presence of tumor on the surface of the skin which is about 1 cm or larger in diameter while the lymph nodes may be enlarged although no metastasis can be seen.
Stage 3 Mycosis fungoides is characterized by generalized erythroderma. The skin surface is nearly covered with tumor while the lymph nodes may be enlarged although there is still no metastasis. Abnormal lymphocytes may be found in the blood although not cancerous.
Stage 4 Mycosis fungoides is divided into stage 4A and stage 4B. Stage 4A is described as Mycosis fungoides with tumors that can cover any amount of the skin surface. This stage can either have cancer that has affected the lymph nodes while the blood may have the presence of cancerous lymphocytes or the stage can also be described as having cancerous lymphocytes in the blood with enlarged lymph nodes while metastasis is absent. Stage 4B is characterized by cancer that has spread to other organs or parts of the body. The lymph nodes may also be enlarged and affected by cancer and cancerous lymphocytes may also be found in the blood.
Mycosis Fungoides Symptoms
Mycosis fungoides is generally presented with plaques or patches covering the skin surface. The patches or skin lesions are asymmetrically distributed in the areas of the skin such as in the buttocks, groin, breasts, hips and lower trunks. The early onset on the other hand may mimic that of other benign skin disorder which makes it difficult to diagnose Mycosis fungoides during the early stage. Mycosis fungoides on the other hand progresses into stages defined by the symptoms of the skin.
Patch phase defined by the development of flat and itchy red patches.
Plaque phase is the evolvement of patches when it becomes more infiltrative.
Mycotic phase is the appearance of raising nodules similar to the appearance of a mushroom.
The exact cause of Mycosis fungoides remains vague while hereditary or genetic cause is not being linked to the incidence of most cases of Mycosis fungoides. It is a non-Hodgkin lymphoma believed to be the result of changes in the DNA which led to lymphoma cells of the normal lymphocytes. The changes in the DNA related to non-Hodgkin lymphoma are usually acquired after birth and are not inherited and usually occur as a person advances in age. This explains the reason behind the incidence of lymphoma in older people although it can also affect children and adolescents.
The treatment of Mycosis fungoides depends on its stage from the time of diagnosis, including the overall health condition of the patient. The treatment plan is tailored according to the needs of the patient and no two patients can exactly have the same treatment plan.
The common treatment of Mycosis fungoides during the early stage may include the following:
- Electron beam radiation
- Application of steroid creams on the skin
- Sunlight or ultraviolet light
Chemotherapy or recombinant alfa interferon may be used in Mycosis fungoides when it is in tumor stage and has failed to respond to skin treatments.
Mycosis fungoides can be disfiguring and is a potential life threat particularly on its advance stage. It is an incurable disease that the main objective of treatment is the quality of life and to make the most of the remission period. The degree of survival of the patient depends on the extent of the spread of the disease from the time it was first diagnosed.