Mycosis fungoides (primary skin lymphoma, MF, Latin mycosis fungoides) is one of the most common chronic skin lymphomas in the population. What are the causes and symptoms of this lymphoma? How is the treatment of mycosis fungoides?
Mycosis fungoidesis a malignant neoplasm that originates in the lymphatic system and is based on the proliferation of T lymphocytes in the patient's skin (CTCL, Cutaneus T-cell Lymphoma).
The first symptom of this disease, which worries patients and prompts them to see a doctor, are very itchy skin lesions in the form of a rash, reddening and peeling of a certain area of the skin, as well as the appearance of blisters and papules on it.
With the advancement of the disease, extra-cutaneous changes develop - enlargement of the lymph nodes and involvement of internal organs. The disease is characterized by many years of course and for a long time it does not cause symptoms other than dermatological changes.
Mycosis fungoides: epidemiology
Primary cutaneous lymphoma is much more common in people over 40, as well as the elderly, men more often than women (2-3: 1). It is a rare malignant neoplasm, with a frequency of about 0.3-0.5 / 100,000 people.
Mycosis fungoides: causes
The causes of mycosis fungoides have not yet been known. However, it is assumed that the development of the disease is influenced by the infection with the virus that causes acute leukemia type T (Human T-cell Leukemia / Lymphoma Virus HTLV-1).
Mycosis fungoides: symptoms and course
- STADIUM 1 (introductory period)
The first stage of mycosis fungoides is characterized by very itchy dermatological lesions that can appear on the skin all over the body, but are most common on the trunk and buttocks, upper thighs and arms.
Plaque areas of red-violet skin with distinct boundaries are typical. Its surface is prone to irritation, dry, tissue paper, wrinkled, with slight lumps and is flaky.
Vesicular or pustular eruptions are rarely observed. The initial period of mycosis fungoides may be even several years.
- STADIUM 2 (infiltration period)
The second stage of cutaneous lymphoma involves further spread of skin lesions and intensification of the itching sensation.
Around the erythematous lesions, which are slightly raised and well demarcated from he althy skin, there are infiltrations of various diameters, often ring-shaped, palpable under the fingers.
You can observe more and more exfoliation of the skin. At this stage of disease advancement, hair loss occurs as a result of the disease becoming affected by the disease and the destruction of the hair follicles.
- STADIUM 3 (lumpy period)
The third stage of mycosis fungoides is manifested by the presence of nodular changes within previous dermatological changes, as well as in he althy skin.
Tumors grow rapidly, have a reddish brown color and ooze surface. They can disintegrate, crack, and on their surface form deep cavities in the epidermis and dermis - erosions and ulcers - which are difficult to heal.
At this stage of advancement of mycosis fungoides, skin lesions are often accompanied by lesions of other organs.
The patient often has palpable enlarged lymph nodes, as well as internal organs affected by the neoplastic process - bone marrow, lungs, spleen, liver, sometimes also the gastrointestinal tract and the central nervous system. The appearance of organ lesions is not favorable.
General symptoms such as weight loss, lack of appetite and malaise can be observed in many patients.
Mycosis fungoides: new division
The literature describes a new division of mycosis fungoides, which is based on the characterization of skin lesions, the area of the skin they cover and the involvement of lymph nodes and internal organs or not.
| Period I A |
Skin lesions occupy less than 10% of the skin surface |
| Period I B |
Skin lesions occupy more than 10% of the skin surface |
| Period II A |
Skin lesions occupy more than 10% of the skin surface |
| Period II B |
Lumpy period |
| Period III |
Erythrodermic changes |
| Period IV A |
Diffuse extensive skin lesions |
| Period IV B |
Diffuse extensive skin lesions |
Mycosis fungoides: diagnosis and diagnosis
Diagnosis of mycosis fungoides only on the basis of blood test results and clinical symptoms is not possible because they are not very specific.
The main problem of the patient who most often turns to their GP are itchy skin lesions.
After examining the patient, the doctor should issue a referral for an appointment with a specialist dermatologist. In order to establish the correct diagnosis, it is necessary to perform a histopathological examination of the section of the affected skin, which in this case is decisive.
It should be noted that tests performed in the early stages of the disease may be negative, and the microscopic image characteristic of mycosis fungoides will appear later and the patient will require another skin biopsy to establish a final diagnosis.
It is important to constantly monitor the patient's condition and perform periodic laboratory tests. If skin lesions change, cover a larger area, grow in thickness - another skin biopsy is recommended.
Lymph nodes should be regularly examined by ultrasound and collected for examination.
Mycosis fungoides: differentiation
The diagnosis of mycosis fungoides can only be established on the basis of a histopathological examination of a skin specimen (skin biopsy).
There are no known pathognomonic dermatological changes typical of this disease, therefore it is so important to perform a differential diagnosis of the disease and exclude other disease entities.
Similar skin lesions as in the discussed primary skin lymphoma, also occur in inflammatory lesions of the skin, drug reactions, atopic dermatitis (AD), psoriasis, plaque psoriasis, lichen planus, and disseminated eczema.
Mycosis fungoides: treatment
The method of treating the lymphoma in question strictly depends on the stage of the disease at which the disease was diagnosed.
In the initial stages of the granuloma (initial period and infiltration period), the therapy usesUVA and UVB ultraviolet radiationin specially prepared lamps.
Sometimes a specialist dermatologist may decideabout adding specialized drugs to phototherapy.
PUVA (Psoralen Ultra-Violet A) therapyis a treatment method that uses UVA radiation with oral administration of drugs (Psoralen), which are photosensitizing, i.e. sensitizing the skin to radiation.
RE-PUVA (Retinoid PUVA)consists in adding an additional drug to PUVA therapy, i.e. retinoids, i.e. acid derivatives of vitamin A.
In the early stages of the disease, you can also use low doses of X-rays, and add interferon alfa to PUVA therapy.
Pharmacological chemotherapy is the treatment of choice in the advanced stage of mycosis fungoides (nodular period). The most commonly used cytostatics are cyclophosphamide, methotrexate, chlorambucil, cisplatin, vincristine, vinblastine, and also bleomycin.
Mycosis fungoides: prognosis
Characteristic for mycosis fungoides is the chronic course of the disease, low malignancy, and therefore long-term survival.
The vast majority of patients remain in the early stage of the disease throughout their lives. However, it should be remembered that there are cases of patients (about 10% of patients) in whom the course of the disease is very rapid and the prognosis in such a case is not favorable.
It is assumed that the survival time of patients diagnosed in the early stage of the disease is approximately 25 years, and those diagnosed in the infiltrative stage of clinical advancement approximately 5 years.
In people diagnosed at the end of the nodular period, the average survival time is only 1-2 years.