Mycosis Fungoides: Causes, Symptoms & Treatment

Mycosis fungoides is a rare tumor disease that originates from degenerate T lymphocytes and manifests primarily in skin tissue. The course of the tumor disease is chronic-progressive and infaust, although the prognosis for mycosis fungoides can be significantly improved by early initiation of therapy.

What is mycosis fungoides?

Mycosis fungoides is the name given to a rare, low-malignant (less malignant) cutaneous T-cell lymphoma that has a chronic progressive course and originates 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, tumor disease of the lymphatic tissue) and has some special forms that entail a significantly less favorable prognosis. Thus, in the so-called mycosis fungoides d’emlée, tumors manifest from the beginning in the skin as well as the mucosa (mouth, nose, pharynx), while in more advanced stages of the disease, the internal organs (especially lymph nodes, liver, spleen) may also be affected. In the so-called Sézary syndrome, a greatly increased concentration of degenerate, atypical T lymphocytes can be detected in the blood in addition (leukemic form of mycosis fungoides).

Causes

Mycosis fungoides originates from one or more degenerate T lymphocytes, which play an important role in the body’s immune defenses 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 persistent, eczema-like skin lesions. The triggering factors for this degeneration process have not yet been clarified. A connection between certain viruses (especially the retrovirus HTLV-1) and mycosis fungoides could not be proven so far. In addition, long-term contact with carcinogenic substances has been found in many affected persons working in the agricultural or metal-processing sector. Chronic inflammation, which causes increased growth of T lymphocytes, is also discussed as a triggering factor for mycosis fungoides.

Symptoms, complaints, and signs

Mycosis fungoides may initially be completely asymptomatic. The first signs are skin changes reminiscent of psoriasis. In addition, painful eczema forms on the skin and scars over the course of the disease. The skin appears dry and irritated, which can result in itching and reddening of the skin. Furthermore, the disease may 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, this can lead to irreversible skin damage. Then, psychological complaints, such as inferiority complexes and social anxiety, often develop as well. Mycosis fungoides progresses progressively, causing ever greater health problems as it progresses. Involvement of the lymphatic system and internal organs leads to cardiovascular problems, gastrointestinal problems, hormonal disorders and other health problems. The quality of life is greatly reduced as a result. If the tumor disease is treated early, the symptoms subside completely within a few weeks to months. In advanced mycosis fungoides, scars as well as surgical scars may remain. Early treatment prevents further symptoms and any late sequelae, with symptoms usually resolving a few weeks after treatment begins.

Diagnosis and course

Mycosis fungoides is usually diagnosed by biopsy (tissue removal) from the diseased areas of skin. If abscess-like collections of lymphoid cells are found in the removed epidermal tissue, mycosis fungoides can be assumed. The diagnosis is confirmed by blood analysis. If an increased lymphocyte concentration and/or an increased number of class E immunoglobulins can be detected, the diagnosis is considered confirmed. Mycosis fungoides has a very slow course and causes only a few characteristic symptoms at the beginning. In general, the tumor disease has an unfavorable prognosis and definite cures are considered extremely rare.If left untreated, the tumor cells in mycosis fungoides spread to the internal organs (liver, spleen) through the blood and lymphatic systems.

Complications

Because mycosis fungoides is a tumor disease, in some cases it can spread to other and especially healthy tissues, causing irreversible damage. For this reason, it is not possible to make a general prediction about the symptoms and course of the disease. However, with early treatment, complications and subsequent damage can be avoided. Those affected suffer mainly from discomfort on the skin. The skin is reddened and usually also affected by an unpleasant itching. The skin is also very dry and can also flake. Many patients feel uncomfortable with the discomfort and suffer from inferiority complexes or lowered self-esteem as a result. In some cases, social exclusion also occurs, leading to depression and other psychological upsets. Mycosis fungoides can be limited and treated by various therapies. The success of the treatment depends strongly on the timing and the extent of the tumor. If necessary, mycosis fungoides also limits the patient’s life expectancy. Chemotherapy in particular can lead to various complications and side effects that significantly reduce the quality of life of the affected person.

When should you see a doctor?

Symptoms such as itching or redness of the skin may indicate a tumor disease that needs to be examined and, if necessary, medically treated. A visit to the doctor 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. Therefore, even at the first suspicion should be spoken with the family doctor. Affected individuals should also consult a dermatologist. Anyone who has already had a tumor is a high-risk patient and should talk to the doctor in charge. The same applies to people with a weakened immune system or other physical conditions that favor the growth of a tumor. Medical help is needed at the latest when complications such as severe pain or hormonal fluctuations are noticed. In addition to the family doctor, an internist or a dermatologist can be consulted. In the case of serious diseases, an orthopedic examination is necessary concomitantly, so that any damage to the bones and joints can be detected and treated.

Treatment and therapy

Therapeutic measures in mycosis fungoides depend on the present stage of development of the tumor disease. At the onset 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 irradiation with long-wave UV-A light. In some cases, irradiation of the localized, atypical skin changes with X-rays is sufficient at this stage. In the advanced stage of development of mycosis fungoides (stage II with plaque and nodule formation to stage IV with involvement of internal organs), PUVA therapy is applied in combination with immunotherapy with interferon alpha. If the lymphatic system and internal organs are involved, additional chemotherapy and radiotherapy are usually indicated for mycosis fungoides. The administered chemotherapeutic agents (mainly cytostatics) destroy the tumor cells and inhibit cell growth. Here, chemotherapy includes several cycles and an individually adapted drug mix (including prednisolone, cyclophosphamide, adriblastin, and vincristine), depending on the tolerance and response to therapy on the part of the person affected by mycosis fungoides.

Outlook and prognosis

The prognosis of mycosis fungoides is based on the stage of the disease. In stage I, cure is possible. The condition may persist for many years and cause persistent physical symptoms. In the course of stage II mycosis fungoides, severe complications occur, such as infestation of internal organs, which eventually lead to death. Quality of life and life expectancy are accordingly severely limited.The prospect of an improvement of the symptoms is no longer given in stage II. 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 places a great deal of strain and stress on those affected and can leave permanent damage. Mycosis fungoides stage III is usually not treatable. Large parts of the skin are diseased, causing patients to suffer from chronic pain and severe malaise, which cannot be reliably treated even with medication. Stage IV mycosis fungoides offers a short life expectancy because large parts of the body are affected.

Prevention

Because the causes of cellular degeneration in mycosis fungoides are not understood, no known preventive measures exist. In general, potential triggers of mycosis fungoides such as carcinogenic substances should be avoided and chronic inflammation should be treated early.

Follow-up

In most cases, very limited or very few measures of direct aftercare are available to the affected person with mycosis fungoides. The affected person should therefore see a physician at a very early stage 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 often is. Therefore, affected individuals should ideally contact a doctor at the first signs of the disease. Patients are often dependent on taking various medications and also on using various ointments and creams. The doctor’s instructions should be followed, with attention paid to regular intake and use and likewise the prescribed dosage. In case of any ambiguity or in case of severe side effects, the person affected with mycosis fungoides should always consult a doctor. Many of those affected are also dependent on psychological help during treatment, whereby the support of one’s own family in particular can have a very positive effect on the further course of the disease. In some cases, mycosis fungoides also reduces the patient’s life expectancy.

What you can do yourself

The possible self-help measures for mycosis fungoides depend on the stage of the tumor disease. In the first stage, symptoms can be relieved by 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 supported. In the advanced stages, mycosis fungoides must be treated in the hospital. The therapy can be supported by the patients with various preparations from naturopathy and homeopathy. The most important self-measure, however, is to keep a diary of complaints and to record any symptoms and complaints in it. Based on this information, the medication can be optimally adjusted. If chemotherapy is administered, the patient must take it easy and at the same time change his diet. The therapy puts a great 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 discomfort and make the therapy as comfortable as possible. Accompanying this, therapeutic counseling is useful. Talking to a psychologist can help to work through the illness and make it easier to deal with.