Mycosis fungoides

Mycosis fungoides

Overview

Mycosis fungoides is a rare type of lymphoma that mainly affects the skin. Lymphoma is a cancer that begins in the lymphocytes, which are a type of white blood cell. The white blood cells are part of the body's germ-fighting immune system.

There are many types of lymphoma. These types are often divided into two categories: Hodgkin lymphoma and non-Hodgkin lymphoma. Mycosis fungoides is a type of non-Hodgkin lymphoma.

Mycosis fungoides got its name from the appearance of some of its skin growths, which may look like mushrooms. Mycosis fungoides got its name from the mushroom like look of some of its skin growths. It is not caused by a fungus. It begins in white blood cells called T cells, also called T lymphocytes. The T cells undergo changes and turn into cancer cells. In mycosis fungoides, the T cells attack the skin. Mycosis fungoides is one of a few types of lymphoma that affect T cells in the skin, called cutaneous T-cell lymphomas.

Mycosis fungoides typically grows slowly. As it grows, it sometimes spreads to the lymph nodes, blood and organs. It is often hard to diagnose early. It usually looks like other skin conditions such as eczema, also called atopic dermatitis, and psoriasis when it starts.

A mycosis fungoides rash can sometimes lessen or go away and come back. This cycle can repeat for months or years before the rash becomes worse. Mycosis fungoides is also easy to miss in tests such as biopsies when it first appears. The sampled cells might look like cells from a typical rash under the microscope. Sometimes cells have to be sampled and studied many times before a diagnosis is reached.

Treatments may include skin creams, light therapy, radiation therapy, immunotherapy and chemotherapy or a combination of several of these options. These treatments can help manage the disease and improve quality of life.

Types

There are several types of mycosis fungoides. These include:

Hypopigmented mycosis fungoides. This type causes scaly patches on the skin that look lighter in color compared with the skin surrounding it. It typically affects children and people with Black and brown skin.

Folliculotropic mycosis fungoides. This type affects the hair follicles. A hair follicle is where hair grows from. This type can look like bumps, acne spots or patches. It typically affects adult men, but children also may be affected.

Granulomatous slack skin. This rare type causes loose, hanging folds in the skin. It can also cause clusters of cells called masses in areas such as the armpits and groin. It typically affects men in their 20s and 30s.

Pagetoid reticulosis. This rare type often shows up as one wartlike or scaly patch, typically on a hand or foot. It usually affects men in their 40s and 50s but can happen at any age.

Poikiloderma mycosis fungoides. This type looks like thin patches of skin that are wrinkled, light and dark, and show blood vessels.

Sometimes people wonder how mycosis fungoides compares to another cutaneous T-cell lymphoma called Sezary syndrome. Like mycosis fungoides, Sezary syndrome also looks like eczema and psoriasis in its early stages. It differs from mycosis fungoides in that it usually expands quickly. In time, Sezary syndrome can cause a rash over the whole body. In addition to the skin, Sezary syndrome often also affects the blood early in the disease.

Symptoms

Signs and symptoms of mycosis fungoides vary depending on the area affected and if the cancer is in early or late stages.

Early-stage symptoms include:

Patches of rash on the skin. The patches usually show up on skin that doesn't get much sun, including the breasts, buttocks and upper thighs. It also can show up on the face, scalp, palms and soles, though that is less common. On white skin, the patches may appear pink or red. On brown skin, they might look pink, red, deep red or purple. On Black skin, they may look pink, purple, gray or dark brown.

The patches can sometimes be lighter or darker than surrounding skin and have a wrinkled pattern.

Patches of scaly skin.

Skin that may itch constantly or from time to time.

Spots on the skin that are in different shapes and sizes.

Late-stage symptoms include:

Thicker areas of raised skin called plaques that cover greater areas of the skin.

Skin growths that are red or purple depending on your skin color.

Lumps that form on the skin that may break open or become infected.

Worsened itching.

Swollen lymph nodes.

Hair loss.

Causes

Mycosis fungoides begins in white blood cells called T cells, also called T lymphocytes. The T cells undergo changes and turn into cancer cells. A cell's DNA holds instructions that tell the cell what to do and when to die. Because of these DNA changes, the cells stop dying. They usually multiply and form a skin patch or a mass. Exactly why the DNA changes happen is not known.

Risk factors

The risk of mycosis fungoides may be higher in:

Older adults. The condition can happen at any age, but it's more common in people in their 50s and older.

People who were born male. The condition is twice as common in people who were born male than it is people who were born female.

Black people. Black people have the highest risk of getting mycosis fungoides and are more likely to get this cancer at an earlier age.

There is no known way to prevent mycosis fungoides.

Complications

Mycosis fungoides may lead to skin infections due to breaks in the skin.

Diagnosis

Getting the right mycosis fungoides diagnosis may take time. Mycosis fungoides can be hard to diagnose because its rash usually looks like the skin conditions eczema and psoriasis when it starts.

The rash tends to stay around. It can sometimes lessen or go away and come back from time to time. This cycle can repeat for months or years before a diagnosis is made. A dermatologist will typically begin with a skin exam. Tests and procedures likely will include blood tests, skin biopsies and imaging tests.

Physical exam

A healthcare professional typically starts with a physical exam to look over your skin for scaly areas, growths and unusual patterns. You likely will be asked how your symptoms have behaved over time. The healthcare professional also will usually check for other signs of mycosis fungoides, such as swollen lymph nodes.

Blood tests

Blood tests such as a complete blood count, T cell receptor and flow cytometry could give information about your condition.

Skin biopsy

A skin biopsy is a necessary part of getting a diagnosis. This procedure collects cells from suspicious tissue on the surface of the body so that they can be tested in a lab. The tests can show whether cancer cells are present in the skin.

A healthcare professional might take the sample of cells with a circular cutting tool. This is called a punch biopsy. For larger areas and growths, the healthcare professional might use a small knife. This is called an excisional biopsy.

Skin biopsies don't always confirm that cancer cells are present. A skin biopsy might show microabscesses in the skin cells under a microscope. Microabscesses are tiny clumps of cells in the top layer of the skin that are not typical cells. Sometimes, sampled cells from a biopsy might look like cells from a typical rash under the microscope. Because of this, you might need more than one skin biopsy over time to arrive at the diagnosis.

Imaging tests

An imaging test takes pictures inside the body. It can show the location and extent of the mycosis fungoides if it involves internal organs. If there is concern that the cancer cells have spread to other parts of the body, your healthcare professional might suggest imaging tests. These might include a computerized tomography (CT) scan, or a positron emission tomography scan (PET).

Testing lymphoma cells in the lab

Cancer cells collected from a biopsy go to a lab for testing. In the lab, specialized tests look for specific things about the cells. When viewed under a microscope, the cancer cells have a different appearance compared with healthy cells. The healthcare team uses the results to find more information about the type of cancer cells that you have.

To decide whether the cells are mycosis fungoides cells, the healthcare professionals in the lab look for:

Proteins on the surface of the cancer cells. The cells can be identified by the presence or absence of certain proteins on their surfaces. These proteins are known as markers.

Changes in the cancer cells' DNA. Cancer happens when cells get changes in their DNA. A cell's DNA holds the instructions that tell a cell what to do. Gene changes cause the cancer cells to stop dying and start multiplying rapidly.

Treatment

Treating mycosis fungoides depends on the symptoms, how much skin is involved, and whether the cancer is limited to the skin or has spread to the lymph nodes, organs or blood. Treatment often involves skin-directed therapies and systemic treatments that work throughout the whole body. People with early-stage mycosis fungoides are usually seen by a dermatologist first. Then they could be referred to a cancer specialist called a hematologist if skin-directed therapies are not working. People with more advanced forms of the cancer are typically seen jointly by a hematologist/oncologist and a dermatologist. A radiation oncologist could be involved in the treatment of mycosis fungoides if radiation therapy is needed.

Treatment may include medicines, radiation therapy, light therapy and bone marrow transplant.

Mycosis fungoides typically cannot be cured, but many treatments can help reduce symptoms and improve the skin. Your treatment plan may include a mix of treatments.

Skin creams and ointments

Some medicines for mycosis fungoides are applied to the skin. The medicines may come in creams, gels and ointments.

Medicines used in this way include:

Steroid medicines. Steroid medicines are commonly used to treat mycosis fungoides. Steroids are either applied to the skin or are in the form of pills. Steroids can help control rash and itchiness.

Chemotherapy medicines. Chemotherapy treats cancer with strong medicines.

Light therapy

Light therapy for mycosis fungoides involves shining a certain kind of light on the skin to kill the cancer cells. During this treatment, you stand in a treatment area while lamps shine on your skin. The treatment often is given a few times a week for several weeks.

Sometimes light therapy also uses medicine to make the cancer cells easier to kill with the light. This is called photodynamic therapy.

Radiation therapy

Radiation therapy treats cancer with powerful energy beams. For mycosis fungoides, the energy beams are most often X-rays or electrons. The treatment might target a small area of cancer on the skin. Or it can be given to all the skin on the body. This is called total skin irradiation.

Medicines in pill form or through a vein

Some medicines for mycosis fungoides are given in pill form or through a vein. Giving the medicine this way means it travels through the body and can treat the cancer wherever it is growing.

Medicines used in this way include:

Chemotherapy. Chemotherapy treats cancer with strong medicines. The medicines kill the cancer cells.

Targeted therapy. Targeted therapy for cancer is a type of treatment that uses medicines that attack specific chemicals in the cancer cells. By blocking these chemicals, targeted treatments can cause cancer cells to die.

Immunotherapy. Immunotherapy for cancer is a treatment with medicine that helps the body's immune system kill cancer cells. The immune system fights off diseases by attacking germs and other cells that shouldn't be in the body. Cancer cells survive by hiding from the immune system. Immunotherapy helps the immune system cells find and kill the cancer cells.

Bone marrow transplant

A bone marrow transplant, also called a bone marrow stem cell transplant, involves putting healthy bone marrow stem cells into the body. These cells replace cells hurt by chemotherapy and other treatments. A bone marrow transplant might be used when mycosis fungoides is far along or comes back after other treatments.

Type of Doctor Department : A dermatologist, a hematologist-oncologist, or a radiation oncologist.