A mycosis fungoides is a rare tumor that originates from degenerated T lymphocytes and manifests itself mainly in the skin tissue. The course of the tumor disease is chronic progressive and infaust, whereby the prognosis of a 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 is chronically progressive and emanates from malignantly degenerated T lymphocytes. The degenerate T lymphocytes affect the skin and cause the skin damage characteristic of mycosis fungoides.

Mycosis fungoides is classified as a non-Hodgkin's lymphoma (NHL, a tumor of the lymphatic tissue) and has some special forms that lead to a much less favorable prognosis. Thus, the so-called mycosis fungoides d'emlée manifests from the beginning tumors in the skin and the mucous membrane (mouth, nose, pharynx), while in more advanced disease stages, the internal organs (especially lymph nodes, liver, spleen) may be affected.

In the so-called Sézary syndrome, a marked increase in the concentration of degenerated, atypical T lymphocytes in the blood is also detectable (leukemic form of mycosis fungoides).

causes

A mycosis fungoides is based on one or more degenerate T lymphocytes, which fulfill an important function in the body's own immune defense and killer cells.

The degenerate T lymphocytes affect the skin (cutaneous T-cell lymphoma) and cause the characteristic symptoms of mycosis fungoides, such as stubborn, eczematous lesions. The triggering factors for this degeneration process have not yet been clarified. A connection between certain viruses (in particular the retrovirus HTLV-1) and a mycosis fungoides has not yet been detected.

In addition, long-term exposure to carcinogenic substances has been identified among many stakeholders in the agricultural or metalworking sector. Chronic inflammation, which causes increased growth of T lymphocytes, is also discussed as a triggering factor for mycosis fungoides.

Symptoms, complaints & signs

A mycosis fungoides can initially run completely symptom-free. The first signs are skin lesions reminiscent of psoriasis. In addition, painful eczema on the skin, which scar in the course of the disease. The skin appears dry and irritated, resulting in itching and redness.

In addition, the disease may cause edema on the skin. These can ignite and in the worst case lead to a bacterial superinfection. If the tumor spreads to healthy tissue, it can cause irreversible skin damage. Then often psychological complaints develop, such as inferiority complexes and social anxieties.

Mycosis fungoides progresses progressively, causing more and more health problems. Involvement of the lymphatic system and the internal organs causes cardiovascular complaints, gastrointestinal complaints, hormonal disorders and other health problems. The quality of life is thereby greatly reduced.

If the tumor disease is treated early, the symptoms disappear completely within a few weeks to months. Scarring and surgical scars may be present in advanced mycosis fungoides. Early treatment prevents further discomfort and possible long-term consequences, with the symptoms usually subside again a few weeks after the start of treatment.

Diagnosis & History

A mycosis fungoides is usually diagnosed by a biopsy (tissue removal) from the diseased skin areas. If epinephrine accumulations of lymphoid cells are detected in the removed epidermis tissue, a mycosis fungoides can be assumed.

The diagnosis is confirmed by a blood analysis. If an increased lymphocyte concentration and / or an increased number of immunoglobulins of class E can be detected, the diagnosis is considered to be assured.

A mycosis fungoides has a very slow course and causes only a few characteristic symptoms at the beginning. In general, the tumor disease has an infamous (unfavorable) prognosis and definite healing is considered extremely rare. Untreated, the tumor cells spread in a Mycosis fungoides on the internal organs (liver, spleen) via the blood and lymphatic system.

complications

Since mycosis fungoides is a tumor disease, it can in some cases also spread to other and above all healthy tissue and cause irreversible damage there. For this reason, no general prognosis about the symptoms and the course of the disease is possible with this disease. However, early treatment can avoid complications and consequential damage.

The affected suffer mainly from complaints on the skin. The skin is reddened and usually also affected by an unpleasant itching. Also, the skin is very dry and can also dandruff. Many patients feel uncomfortable with the symptoms and suffer from inferiority complexes or reduced self-esteem.

In some cases it also leads to social exclusion and thereby to depression and other mental upsets. Through various therapies, the mycosis fungoides can be restricted and treated. The success of treatment depends greatly on the time and on the severity of the tumor.

If necessary, mycosis fungoides also limits the life expectancy of the patient. Especially with chemotherapy, various complications and side effects can occur, which significantly reduce the quality of life of the person affected.

When should you go to the doctor?

Symptoms such as itching or flushing may indicate a tumor disease that needs to be investigated and medically treated if necessary. A visit to a doctor is recommended if the symptoms persist for longer than a few days or rapidly increase in intensity. If the disease is detected early, the prognosis is good. Therefore, should be spoken at a first suspicion with the family doctor. Affected individuals should also visit a dermatologist. Anyone who has ever had a tumor is one of the risk patients and should talk 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 hormone fluctuations are noticed, medical attention is needed. In addition to the family doctor, an internist or a dermatologist can be visited. In the case of serious illnesses, an orthopedic examination is necessary to detect and treat any damage to the bones and joints.

Treatment & Therapy

The therapeutic measures depend on a mycosis fungoides from the present developmental stage 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) as well as cortisone ointments are usually used to treat the atypical skin changes.

As part of this therapy, psoralen (photosensitizing substance) is applied locally or orally for several hours before irradiation with long-wave UV-A light. In some cases, irradiation of the localized, atypical lesions with X-rays is sufficient at this stage. In the advanced development stage of mycosis fungoides (stage II with plaque and nodule formation to stage IV with involvement of the internal organs), the PUVA therapy is used in combination with an immunotherapy with interferon-alpha.

If the lymphatic system and the internal organs are involved, additional chemo- and radiotherapeutic measures are usually indicated in case of mycosis fungoides. The administered chemotherapeutic agents (especially cytostatics) destroy the tumor cells and inhibit cell growth.

Depending on the tolerability and the response to the therapy by the person affected by mycosis fungoides, chemotherapy includes several cycles as well as a customized medication mix (eg prednisolone, cyclophosphamide, adrenoblastin, vincristine).

Outlook & Forecast

The prognosis of mycosis fungoides is based on the stage of the disease. In stage I, a cure is possible. Suffering can persist for many years and cause prolonged physical discomfort. In the course of mycosis fungoides stage II, severe complications such as the involvement of internal organs occur, which eventually lead to death. Quality of life and life expectancy are accordingly severely limited.

The prospect of an improvement of the complaints is no longer present in stage II. Only chemotherapy is considered for lymph node and organ involvement in an advanced stage of the disease. However, the treatment also damages the healthy tissue and causes complications such as hair loss, gastrointestinal disorders and an increased susceptibility to infection.

Chemotherapy is a great effort and strain for those affected and can leave lasting damage. Mycosis fungoides stage III is usually untreatable. Large parts of the skin are affected, which causes the patient to suffer from chronic pain and a strong discomfort, which can not be reliably treated with medication. The Mycosis fungoides stage IV offers a low life expectancy because large parts of the body are affected.

prevention

Since the causes of the cell degeneration process in a mycosis fungoides are not clear, there are no known preventive measures. In general, possible triggers of a mycosis fungoides like carcinogenic substances should be avoided and chronic inflammation should be treated early.

aftercare

In most cases, only very limited or very few immediate follow-up measures are available to the person concerned with mycosis fungoides. The affected person should therefore go to a doctor very early to prevent the occurrence of further complications and complaints. The sooner this tumor is detected and treated, the better is often the further course of the disease.

Therefore, sufferers should ideally contact a doctor at the first sign of the disease. Patients often rely on the use of various medications and also on the use of various ointments and creams. The doctor's instructions should be followed, taking care to ensure that they are regularly taken and used, as well as the prescribed dosage.

In case of ambiguity or severe side effects, the person concerned should always consult a doctor for mycosis fungoides. Many of those affected are also dependent on psychological help during the treatment, with support from their own families in particular having a very positive effect on the further course of the disease. In some cases, mycosis fungoides also reduces the life expectancy of the patient.

You can do that yourself

The possible self-help measures depend on the stage of the tumor disease in a mycosis fungoides. In the first stage the complaints can be alleviated by cortisone ointments and similar preparations. Associated with an individual diet and moderate exercise, the growth of tumors can be slowed down and the healing process can be assisted.

In advanced stages, a mycosis fungoides must be treated in the hospital. The therapy can be supported by the patients by different preparations from the naturopathy and the homeopathy. The most important thing, however, is to create a complaint diary and record any symptoms and complaints. Based on this information, the medication can be optimally adjusted. If chemotherapy is used, the patient must take care and change his diet at the same time.

The therapy is a great burden on the body and the psyche, which is why comprehensive preparatory measures must be taken. The doctor, together with the patient, will take appropriate steps to reduce the discomfort and make therapy as comfortable as possible. Accompanying this, a therapeutic consultation makes sense. In conversation with a psychologist, the disease can be worked up and the handling of it easier.

Tags: 
  • treatments 
  • pathogen 
  • teething 
  • to travel 
  • babies and toddlers 
  • Top