What is Fungal Mycosis?
Mycosis fungoid – a malignant tumor of lymphoid tissue, characterized by excessive proliferation of T-helper cells in the skin. In the later stages, the lymph nodes and internal organs are affected. Sesari syndrome (Sesari reticulose, reticular erythroderma) is a type of fungal mycosis characterized by erythroderma with desquamation and itching, melasma, lymphadenopathy, alopecia, and leukocytosis and the presence of atypical macrophages in the blood.
Usually, fungal mycosis begins with persistent scaly patches that are poorly amenable to local treatment with emollients and steroid preparations. On average, it takes 7 years from the time of onset of development of a skin lesion before diagnosis. In this early phase, the disease is often diagnosed as plaque parapsoriasis. Over time, the spots thicken and become plaques. Skin tumors are gradually formed and lymph nodes may be involved. Lesions of the visceral organs in this poorly differentiated lymphoma appear late. The average life expectancy in patients with this disease at the stage of spots and plaques is 12 years, at the tumor stage – 5 years, at the stage of lesions of the lymph nodes and visceral organs – 3 years.
Both sexes are sick with fungal mycosis, men more often. The disease occurs mainly at the age of 40-60 years. The frequency of 0.29 cases per 100,000 population.
In recent years, cases of registration of fungal mycosis have become frequent, possibly due to improved diagnostics, especially in the early stages of the disease.
Causes of Fungal Mycosis
The reasons for the development of fungal mycosis is still not clear. To date, the motives of uncontrolled T-cell proliferation are not known. It is assumed that the occurrence of fungal mycosis may be associated with a persistent viral infection. Thus, in a number of works, it has been reported that, in a primary cell culture obtained from a lymph node of a patient in the infiltrative-plaque stage of fungal mycosis, viral particles morphologically characteristic of retroviruses of type C. In addition, in DNA of primary cell cultures obtained from patients with fungal mycosis, The presence of a sequence related to the gag gene HTLV-1 was detected.
A certain role in the occurrence of malignant lymphomas is assigned to genetic factors. It is proved that the development of various types of solid tumors and leukemias is associated with the appearance and further increase of chromosomal abnormalities. In recent years, publications have appeared on chromosomal rearrangements in GM, which contribute to the activation of oncogenes and the inactivation of tumor suppression genes during the development of the lymphoproliferative process. Currently, the literature widely discusses the position that significant pathogenetic factors of GM can be carriers of xenogenic information, leading to pathological disruptions in the functioning of various systems and cells of the macroorganism. First of all, such changes were observed on the part of immunocompetent cells, epidermocytes, macrophage elements of the skin, receptor apparatus of the skin and production of cytokines. Available in the domestic and foreign literature information about the state of immune homeostasis strongly suggests persistent pathological changes in both cellular and humoral levels of immunity. Analysis of the immune status showed that in the majority of patients with hypertension, with normal numbers in the vascular bed of B-lymphocytes, the average T-cell content was significantly reduced compared with healthy donors. A decrease in both T-suppressor and T-helper lymphocytes was noted, and the number of the latter decreases more significantly. Relative lymphopenia is considered by many authors as the most frequent hematological sign of GM. Others, in addition, note the presence of absolute lymphopenia in the tumor stage of the disease. The functional activity of lymphocytes in patients with GM, according to several authors, is reduced. Others report no significant differences in the nature of the proliferation of peripheral blood lymphocytes of patients and healthy individuals. It has been established that the course of the pathological process in GM is accompanied by a decrease in the activity of natural killer cells.
The results of studies on the state of the humoral immunity are contradictory. In a number of studies, no significant impairments in the production of immunoglobulin B cells have been identified. However, there are indications of the presence of overt IgM- and latent IgA- and IgG-deficiency of antibody in GM. Other researchers found an increase in the blood of GM patients with IgA, IgM, as well as a significant increase in IgG concentration. An increase in the level of immunoglobulins is explained by the helper phenotype of the tumor substrate in GM. Thus, changes in the immune status of patients with GM are characterized by significant variability and the absence of signs that have absolute diagnostic value.
Pathogenesis during Fungal Mycosis
More often than other forms, there is a form of Alibert-Bazin, which has three stages:
- plaque (infiltrative),
The erythematous stage is often polymorphic and resembles many dermatoses, such as: parapsoriasis, pruritus, lichen planus, herpetiform dermatosis, etc. Rashes are localized on the body, the extensor surfaces of the limbs, can merge with each other, capturing large areas of skin. Rash may be preceded by severe itching. The rash has the appearance of yellowish-pink or bluish-purple spots with a slight grayish peeling. Over the course of several months or several years, the disease goes into a plaque (infiltrative) stage. At this stage, the spots infiltrate and thicken, they grow around the periphery, itching becomes more painful. In addition, the spots become similar to orange peels due to saturated brick-red, purple color. When plaques undergo a reverse development, in their place various shapes are formed in the form of garlands, arcs, and rings. Sometimes these first two stages are called the premotic period, or premotic.
The stage of tumors – the third, the last stage – is the most characteristic. At this stage, the elements become the size of an orange and more and become reddish-bluish or reddish-yellow. Such tumors can simultaneously coexist with plaque infiltrates.
When fungal mycosis increases lymph nodes, and in all three stages. Usually, the inguinal and femoral lymph nodes are first involved in the process. There are two more rare forms of the disease. This is a form of Widal-Brock, in which the first stages are absent and on the apparently healthy skin there is a sudden appearance of tumors. Gallop-Bénier form is characterized by the development of universal exfoliative erythroderma.
Symptoms of Fungal Mycosis
The most common form of Alibert – Bazin, which is divided into three stages: erythematous, plaque (infiltrative) and tumor. In the erythematous stage, yellowish-pink or bluish-purple spots with a slight grayish peeling appear, accompanied by very severe itching that may precede the rash. Initially, the rash located on the body, extensor surfaces of the limbs, and then, merging, can capture significant areas of the skin. This stage often has a polymorphic pattern and can resemble many dermatoses (parapsoriasis, pruritus, lichen planus, dermatitis herpetiformis, etc.). Some authors believe that a number of dermatoses can transform into fungal mycosis. Others believe that the erythematous stage of a fungal mycosis may clinically resemble eczema, psoriasis, and other dermatoses. Unnoticed, after various periods (from several months to several decades), a patchy (infiltrative) stage occurs in which infiltration and thickening of spots occur, their growth along the periphery; itching becomes even more painful. Infiltrative spots become more saturated brick-red, purple color, resembling orange peels. As a result of the regression of plaques, figures are formed in the form of garlands, arcs, and rings. A number of authors call the first two stages of premosis, or zremikotichesky period. The last stage is more characteristic – the stage of tumors, in which the elements reach the size of an orange and more and have a reddish-yellowish or reddish-bluish color. These “tomatoes” can occur simultaneously with plaque infiltrates.
In all three stages, an increase in lymph nodes is noted. Initially, the process involves the inguinal and femoral hymphatic nodes.
The Vidal-Brock form is much less common, with somatic “premotic” periods and on the apparently spongy skin tumors suddenly form, and the erythrodermic Gallop-Bénier form, which is characterized by the development of universal exfoliative erythrodermia (hyperemic, skiltyrirovannaya skin is covered with large plate.
Treatment of Fungal Mycosis
There is an opinion that with the initial, favorably flowing forms of fungal mycosis, it is advisable to prescribe a conservative general strengthening treatment (vitamins of group B, vitamin C) and outwardly steroid ointments. It is also shown spa treatment.
In more pronounced stages of the disease, it is preferable to prescribe a combination therapy: cytotoxic agents, antitumor antibiotics. It is advisable to combine prospidin with corticosteroids.
In these cases, the effect occurs faster with lower doses of drugs, and therefore the number of side effects and complications decreases.
In the tumor stage of a fungal mycosis, radiotherapy is used. Outwardly prescribed means to reduce itching, including corticosteroid ointment.
With modern methods of treatment of fungal mycosis in most cases, long-term remission can be achieved. In these cases, death may occur as a result of complications or intercurrent diseases. In the tumor stage, the prognosis is already.