What is Mycosis Fungoides & How is it Treated?

Cancer is one of the most dreaded diseases that the mankind has ever encountered. Since many of its types doesn’t have a complete cure, even the very name of cancer shudders the patients. Amongst the different types of cancers, one of the most common type is blood cancer. When the blood cells suffer from malignancy, it causes blood cancer. Mycosis Fungoides is a type of blood cancer that many patients suffer from.

Mycosis Fungoides is a type of lymphoma or the lymphoid system cancer. When lymphoma or tumours and cancerous cells develop in the T-cells or a particular type of white blood cells in the body, it is called the cutaneous T-cell lymphoma.

Mycosis Fungoides is the most common type of cutaneous T-cell lymphoma. In this condition, the skin is affected by the cancerous characteristics that are associated with different kinds of lesions in the skin. Though this type of cancer is affected on the skin, the skin cells are not cancerous. It is only because the T-cells accumulate in the skin in an abnormal way. The growth of these T-cells is uncontrolled, excessive and abnormal and due to the interaction of the skin cells with these cancerous T-cells, the various skin lesions are visible.

The naming of Mycosis Fungoides is quite misleading and it has nothing to do with fungal infection. It is an indolent condition, which means that the mycosis fungoides may persist in the same stage or condition for years, sometimes over a decade. The progression into another stage is very slow i.e., the skin patches slowly become thicker and lead to plaques and then turn into tumours.

In Mycosis Fungoides, the signs and symptoms are mostly associated with the skin. However, the cancer may spread into other parts of the body such as the liver, gastrointestinal system, spleen or brain.

The signs and symptoms of Mycosis Fungoides vary from stage to stage. These are –

• Signs and Symptoms of Stage I Mycosis Fungoides:
  • Pruritus or itching
  • Erythematous or red, pink, scaly and flat patches on skin
  • Insomnia.

In most cases, the patches or lesions occur in upper thighs, abdomen, breasts and buttocks. In some cases these patches and lesions are found to be disappearing over time. Sometimes they also lead to the next stage of the condition, converting the patches into plaques and tumours.

• Signs and Symptoms of Stage II Mycosis Fungoides:
  • Circular or oval bluish red plaques
  • Plaques are small in the initial stage
  • Gradually the plaques become elevated
  • Over time the plaques run together, resembling exfoliative dermatitis.

During this stage, another symptom may sometimes be found and it is the abnormal development of dark coloured fatty tissue and macrophages cells. Lymphadenitis or inflammation of the lymph nodes may also be seen at this stage.

• Signs and Symptoms of Stage III Mycosis Fungoides:
  • Rounded, lobulated and mushroom shaped tumours appear
  • These ulcerated tumours are reddish brown or bluish in colour
  • Thick skin layer
  • Necrosis or skin cell death by infiltration of lymphoid cells in the lower skin layer
  • Infiltration of lymphoid cells in upper skin layer.
• Signs and Symptoms of Stage IV Mycosis Fungoides:
  • Disorder spreads throughout body
  • Intestinal ulcer
  • Weakness
  • Enlarged liver and spleen
  • Temperature elevation
  • Dysphagia or difficulty in swallowing and coughing
  • Anaemia
  • Weight loss
  • Malaise or feeling of ill health
  • When brain is affected, pain in eye and vision problems may occur.

The causes of Mycosis Fungoides are unknown. There is no evidence that this condition is genetic or hereditary or can be passed on to the offspring. No link with genetic makeup has ever been found out for Mycosis Fungoides. In fact, environmental condition or association with any micro-organism is also not found.

What causes mycosis fungoides is not known. However, whatever the cause may be, there is no evidence reported that this condition is contagious. This means that mycosis fungoides cannot be transmitted through saliva, touch, body fluids and blood.

Mycosis Fungoides is not a cancer of the skin cells. This means that the skin cells are not malignant. Rather, it is malignancy of the cutaneous T-cells, a type of white blood cells. These cutaneous T-cells are related to the skin i.e., in a dynamic manner, these cells are biologically and biochemically associated with the skin. Hence, due to the unusual expression of these malignant cutaneous T-cells, the disorders and symptoms are expressed on the skin in the form of lesions.

About 70% of all Cutaneous T-cell lymphomas are Mycosis Fungoides. In the United States alone, about 6.4 cases per million people are reported to have Mycosis Fungoides. It is not known why males have a higher rate of being affected by this disease, but reports prove that the males are affected twice the number of females. Worldwide, 1 out of 100,000 to 350,000 people are reported to be affected by this condition every year.

Mycosis Fungoides can occur at any age; but it is more common in the age group between 20 and 60. However, instances are there in which the infants and the people aged more than 80 have been affected by this disease.

Certain races are there that have been reported to have suffered more from this disease than others and they include –

• People belonging to the Saharan African region, more than those from the European lineage
• People of Arabian countries are affected more than the non-Arabian countries.

Mycosis Fungoides is an incurable condition. However, if the condition is diagnosed in an early stage, there are chances of complete cure. The prognosis and mortality rate of Mycosis Fungoides depends entirely on –

• The stage of the disease, when it is diagnosed
• Whether the patient has other cutaneous diseases
• The extent of the lesions.

Despite treatments and therapies, the malignant cells of Mycosis Fungoides infiltrate peripheral blood vessels and lymph nodes. It can progress into tumours and also spread in the internal organs and lymph nodes, resulting in a debilitating condition.

If Mycosis Fungoides reaches the final stage, it can result in systemic infection and suppress the immunity. This can cause death. Mortality associated with Mycosis Fungoides is also caused by the secondary malignancies that this condition leads to.

A reliable diagnosis of mycosis fungoides often takes many years. The reason behind this is that the skin lesions are often mistaken to be psoriasis and eczema. The doctor will take into account a complete physical examination and medical history. Biopsy of the skin tissue will be performed. However, several biopsy tests are to be performed to confirm the condition, as in an early stage, it is quite difficult to distinguish the cutaneous T-cells from the other skin conditions.

Along with the biopsy test, there are some other tests that must also be performed and these are –

• Peripheral blood smear or checking if the blood cells look normal, under the microscope.
• CT scan or computerized tomography
• Chest X-ray
• Immunophenotyping
• Flow cytometry
• PET scan or positron emission tomographic scan
• Biopsy of the lymph nodes in case the lymph nodes are found to be enlarged and if there is a clinical suspicion of Mycosis Fungoides.

If these tests diagnose the condition as mycosis fungoides, the next step that is very important is to stage the disease. There are a number of tests that are performed to stage the condition.

There are 3 stages of the disease. Depending on the skin lesions and their extent, the stage of the disease can be determined. These are –

• Premycotic: Itchy, scaly and red lesions on the skin
• Mycotic: Infiltrative plaques
• Tumorous: Dense infiltrate of lymphocytes.

Depending on the stage of the condition, the treatment will be performed and the prognosis would be determined.

Treatment of Mycosis Fungoides is not curative. They are focussed on two different stages. These are –

• Symptomatic Treatment of Mycosis Fungoides: To prevent the irritation and dryness of the skin, the doctor will refer to the moisturizing regime for the skin and prescribe using steroid ointments and creams. For the topical treatment, topical steroid and retinoids are used. Along with this, topical chemotherapy is also done. On the skin lesions and patches, UVA and UVB treatment is applied. Phototherapy or PUVA or psoralen and UVA treatment is also done by applying electron beam radiation on the patches and plaque on the total body.
• Systemic Treatment for Mycosis Fungoides: In cases, where the condition of the patient has only worsened, systemic treatments such as Extracorporeal Photopheresis are applied. Oral retinoids, chemotherapy agents such as Gemcitabine, Pentostatin, Doxorubicin etc. are given. The other systemic treatments include histone deacetylase inhibitor treatment and fusion toxin treatment.

If Mycosis Fungoides is not diagnosed at an early stage, the malignancy may spread to the other organs. Thus, many complications may occur, such as –

• High-output cardiac failure
• Infection
• Secondary malignancies like colon cancer, melanoma and other skin cancers
• Oedema
• Anaemia of chronic disorders
• Non-Hodgkin’s lymphomas
• Hodgkin’s lymphoma.

Since the causes of this disease is not clearly known, it is imperative that if any kind of skin lesion or other symptoms are encountered, medical attention is sought after immediately. Otherwise, the condition might lead to other complications.