Mycosis fungoides is a rare tumor disease that starts from degenerated T lymphocytes and manifests itself primarily in the skin tissue. The course of the tumor disease is chronically progressive and infaust, whereby the prognosis in the case of mycosis fungoides can be significantly improved by an early start of therapy.
What is mycosis fungoides?
Mycosis fungoides is a rare, low-grade (less malignant) cutaneous T-cell lymphoma that has a chronic, progressive course and originates from malignant T-lymphocytes. The degenerated T-lymphocytes attack the skin and cause the skin damage that is characteristic of mycosis fungoides. See eshaoxing for Ketoacidosis Definition and Meaning.
Mycosis fungoides is one of the non-Hodgkin lymphomas (NHL, tumor disease of the lymphatic tissue) and has some special forms that result in a significantly less favorable prognosis. In so-called mycosis fungoides d’emlée, tumors in the skin and mucous membranes (mouth, nose, pharynx) manifest themselves from the start, while in more advanced stages of the disease the internal organs (especially lymph nodes, liver, spleen) can also be affected.
In the so-called Sézary syndrome, there is also a greatly increased concentration of degenerated, atypical T lymphocytes in the blood (leukemic form of mycosis fungoides).
Causes
Mycosis fungoides starts from one or more degenerated T-lymphocytes, which fulfill an important function in the body’s own immune system as defense or killer cells.
The degenerated T-lymphocytes attack the skin (cutaneous T-cell lymphoma) and cause the symptoms characteristic of mycosis fungoides, such as stubborn, eczema-like skin changes. The triggering factors for this degeneration process could not be clarified so far. A connection between certain viruses (especially the retrovirus HTLV-1) and mycosis fungoides has not yet been proven.
In addition, long-term contact with carcinogenic substances was found in many of those affected who work in agriculture or metalworking. Chronic inflammation, which causes increased growth of T-lymphocytes, is also discussed as a triggering factor for mycosis fungoides.
Symptoms, Ailments & Signs
Mycosis fungoides can initially be completely symptom-free. The first signs are skin changes reminiscent of psoriasis. In addition, painful eczema forms on the skin, which scars as the disease progresses. The skin appears dry and irritated, which can result in itching and reddening of the skin.
The disease can also cause edema on the skin. These can become inflamed and, in the worst case, lead to a bacterial superinfection. If the tumor spreads to healthy tissue, it can cause irreversible skin damage. Then mental problems often develop, such as inferiority complexes and social anxiety.
Mycosis fungoides progresses progressively, so it causes ever greater health problems as it progresses. If the lymphatic system and internal organs are involved, cardiovascular problems, gastrointestinal problems, hormonal disorders and other health problems arise. This greatly reduces the quality of life.
If the tumor disease is treated early, the symptoms subside completely within a few weeks to months. In the case of advanced mycosis fungoides, scars and surgical scars can remain. Early treatment prevents further symptoms and any long-term effects, with the symptoms usually disappearing a few weeks after the start of treatment.
Diagnosis & History
Mycosis fungoides is usually diagnosed based on a biopsy (tissue removal) from the diseased areas of the skin. If abscess-like accumulations of lymphoid cells are found in the removed epidermal tissue, mycosis fungoides can be assumed.
The diagnosis is confirmed by a blood analysis. If an increased lymphocyte concentration and/or an increased number of class E immunoglobulins can be detected, the diagnosis is considered to be confirmed.
Mycosis fungoides has a very slow course and initially causes only a few characteristic symptoms. In general, the tumor disease has an infaust (unfavorable) prognosis and definitive healing is considered extremely rare. If left untreated, the tumor cells in mycosis fungoides spread to the internal organs (liver, spleen) via the blood and lymphatic system.
Complications
Since mycosis fungoides is a tumor disease, in some cases it can also spread to other, especially healthy, tissue and cause irreversible damage there. For this reason, it is not possible to make a general prediction about the symptoms and the course of the disease with this disease. With early treatment, however, complications and consequential damage can be avoided.
Those affected suffer primarily from skin complaints. The skin is red and usually also affected by an unpleasant itching. The skin is also very dry and can also flake. Many patients feel uncomfortable with the symptoms and suffer from inferiority complexes or reduced self-esteem.
In some cases, social exclusion also occurs and this leads to depression and other mental disorders. Various therapies can be used to limit and treat mycosis fungoides. The success of the treatment depends heavily on the point in time and the severity of the tumor.
The life expectancy of the patient may also be limited by the mycosis fungoides. Chemotherapy in particular can lead to various complications and side effects that significantly reduce the quality of life of those affected.
When should you go to the doctor?
Symptoms such as itching or reddening of the skin may indicate a tumor that needs to be examined and, if necessary, treated medically. A doctor’s visit is recommended if the symptoms persist for more than a few days or rapidly increase in intensity. If the disease is detected early, the prognosis is good. That’s why you should talk to your family doctor as soon as you have a first suspicion. Affected people should also see a dermatologist. Anyone who has ever had a tumor is one of the risk patients and should speak to the responsible doctor.
The same applies to people with a weakened immune system or other physical ailments that promote the growth of a tumor. At the latest when complications such as severe pain or hormonal fluctuations are noticed, medical help is needed. In addition to the family doctor, an internist or a dermatologist can be consulted. In the case of serious illnesses, an accompanying orthopedic examination is necessary so that any damage to the bones and joints can be identified and treated.
Treatment & Therapy
In the case of mycosis fungoides, the therapeutic measures depend on the stage of development of the tumor disease. At the beginning of mycosis fungoides (stage I with enlarged lymph nodes and eczema-like, scaly patches), photochemotherapy or PUVA (psoralen plus UV-A) and cortisone ointments are usually used to treat the atypical skin changes.
As part of this therapy, psoralen (photosensitizing substance) is applied locally or orally a few hours before exposure to long-wave UVA light. In some cases, it is sufficient at this stage to irradiate the localized, atypical skin changes with X-rays. In the advanced stage of development of mycosis fungoides (stage II with plaque and nodule formation to stage IV with involvement of the internal organs), PUVA therapy is used in combination with immunotherapy with interferon alpha.
If the lymphatic system and internal organs are involved, additional chemotherapeutic and radiotherapeutic measures are usually indicated for mycosis fungoides. The administered chemotherapeutics (above all cytostatics) destroy the tumor cells and inhibit cell growth.
Depending on the tolerability and the response to the therapy on the part of the person affected by mycosis fungoides, chemotherapy includes several cycles and an individually adapted drug mix (including prednisolone, cyclophosphamide, adriblastine, vincristine).
Outlook & Forecast
The prognosis of mycosis fungoides depends on the stage of the disease. In stage I, a cure is possible. The condition can persist for many years and cause persistent physical discomfort. In the course of mycosis fungoides stage II, serious complications such as the infestation of internal organs occur, which ultimately lead to death. Quality of life and life expectancy are therefore severely restricted.
In stage II, there is no longer any prospect of an improvement in the symptoms. Only chemotherapy can be considered for lymph node and organ involvement in an advanced stage of the disease. However, the treatment also damages healthy tissue and results in complications such as hair loss, gastrointestinal disorders and an increased susceptibility to infections.
Chemotherapy is a great effort and burden for those affected and can leave permanent damage. Mycosis fungoides stage III is usually not treatable. Large parts of the skin are diseased, causing the patients to suffer from chronic pain and severe discomfort, which cannot be reliably treated with medication. Stage IV mycosis fungoides has a short life expectancy because large parts of the body are affected.
Prevention
Since the causes of the cell degeneration process in mycosis fungoides have not been clarified, there are no known preventive measures. In general, possible triggers of mycosis fungoides such as carcinogenic substances should be avoided and chronic inflammation should be treated at an early stage.
Aftercare
In most cases, those affected by mycosis fungoides have only very limited or very few direct follow-up measures available. The affected person should therefore consult a doctor very early on in order to prevent the occurrence of further complications and symptoms. The earlier this tumor is detected and treated, the better the further course of the disease.
Therefore, those affected should ideally contact a doctor at the first sign of the disease. Patients are often dependent on taking different medications and also on the use of different ointments and creams. The instructions of the doctor should be observed, whereby regular intake and use as well as the specified dosage must be observed.
If anything is unclear or if there are severe side effects, the person affected by Mycosis fungoides should always consult a doctor. Many of those affected also depend on psychological help during treatment, with the support of their own family in particular having a very positive effect on the further course of the disease. In some cases, mycosis fungoides also reduces the patient’s life expectancy.
You can do that yourself
In the case of mycosis fungoides, the possible self-help measures depend on the stage of the tumor disease. In the first stage, the symptoms can be alleviated with cortisone ointments and comparable preparations. Combined with an individual diet and moderate exercise, the growth of the tumors can be slowed down and the healing process can be supported.
In the advanced stages, mycosis fungoides must be treated in the hospital. The therapy can be supported by the patient with various preparations from naturopathy and homeopathy. However, the most important personal measure is to keep a complaints diary and record any symptoms and complaints in it. This information can be used to optimally adjust the medication. If chemotherapy is carried out, the patient must take it easy and change their diet at the same time.
The therapy puts a lot of strain on the body and the psyche, which is why comprehensive preparatory measures must be taken. Together with the patient, the doctor will take appropriate steps to reduce the symptoms and make the therapy as pleasant as possible. Accompanying this, a therapeutic consultation is useful. In a conversation with a psychologist, the illness can be worked through and dealing with it can be made easier.
