Fungoid mycosis

Description:

Mycosis fungoid - the malignant tumor of an adenoid tissue which is characterized by excess proliferation T-helperov in skin. At late stages lymph nodes and internals are surprised. Cesaria's syndrome (Cesaria a reticulosis, an erythrosis reticular) - the kind of fungoid mycosis which is characterized by an erythrosis with a peeling and an itch, a melanoderma, a lymphadenopathy, an alopecia, and also a leukocytosis and existence in blood of atypical macrophages.

Usually fungoid mycosis begins with the resistant shelled spots which will badly respond to topical treatment by means of emollients and steroid drugs. On average from time of the beginning of development of skin defeat before diagnosis there pass 7 years. In this early phase the disease is often diagnosed as a blyashechny parapsoriasis. Over time spots are condensed and become plaques. Skin tumors are gradually formed and lymph nodes can be involved. Defeats of visceral bodies at this low-differentiated lymphoma appear late. Average life expectancy at patients with this disease at a stage of spots and plaques makes 12 years, at a tumor stage - 5 years, at a stage of damage of lymph nodes and visceral bodies - 3 years.

Both floors have fungoid mycosis, men are slightly more often. The disease meets preferential at the age of 40-60 years. Frequency. 0,29 cases on 100 000 population.

In recent years cases of registration of fungoid mycosis became frequent, perhaps, due to diagnosis improvement, especially in early stages of a disease.

Symptoms of Fungoid mycosis:

Most often Aliber's form - Bazena who is subdivided into three stages meets: erythematic, blyashechny (infiltrative) and tumoral. In an erythematic stage there are flesh-colored or cyanotic-crimson spots with an easy grayish peeling which are followed by very severe itch which can precede rashes. In the beginning rashes are located on a trunk, extensor surfaces of extremities, and then, merging, can occupy considerable sites of an integument. This stage quite often has a polymorphic picture and can remind many dermatosis (a parapsoriasis, a prurigo, red flat deprive, a herpetiform dermatosis, etc.). One authors consider that a number of a dermatosis can be transformed to fungoid mycosis. Others believe that the erythematic stage of fungoid mycosis can clinically remind eczema, psoriasis and other dermatosis. Imperceptibly, through various terms (from several months to several decades), there is a blyashechny (infiltrative) stage in which there are an infiltration and consolidation of spots, their growth on the periphery; the itch becomes even more painful. Infiltrative spots gain more saturated brick-red, crimson color, reminding orange crusts. As a result of regress of plaques figures in the form of garlands, arches, rings are formed. A number of authors call the first two stages premikozy, or the zremikotichesky period. The last stage - a stage of tumors at which elements reach the sizes of orange and more is more characteristic and have reddish-yellowish or reddish and cyanotic color. These "tomatoes" can meet along with infiltrates of a blyashechny stage.

In all three stages note a hyperadenosis. In the beginning inguinal and femoral gimfatichesky nodes are involved in process.

Meet Vidalya-Brock's form much less often, at sotory there are no "premikotichesky" periods and on probably shchorovy skin tumors, and an erythrodermic form of Gallopo-Benye of which development of a universal exfoliative erythrosis is characteristic are suddenly formed (hyperemic, shchfiltrirovanny skin is covered with macrolaminar scales).

Reasons of Fungoid mycosis:

The reasons of development of fungoid mycosis are still not clear. So far incentive motives of uncontrollable T-cellular proliferation are not known. It is supposed that developing of fungoid mycosis can be connected with a persistent viral infection. So, in a number of works it is reported about identification in primary cellular culture received from the patient's lymph node in the infiltrative blyashechnoy of a stage of fungoid mycosis, virus particles morphologically characteristic of Page retroviruses Besides, in DNA of primary cellular cultures received from patients with fungoid mycosis existence of the sequence, related to a gene of gag HTLV-1 is revealed.

A certain part in emergence of malignant lymphoma is assigned to genetic factors. It is proved that development of different types of solid tumors and leukemias is connected with emergence and further increase of chromosomal anomalies. In recent years there were publications about chromosomal reorganizations at hypermarkets which promote activation of oncogenes and an inactivation of genes of tumoral suppression at development of limfoproliferativny process. Now in literature the provision that significant pathogenetic factors of hypermarket can be carriers of the xenogenic information leading to pathological failures in functioning of various systems and cells of a macroorganism is widely discussed. First of all such changes were observed from immunocompetent cells, epidermotsit, macrophagic elements of skin, the receptor device of skin and products of cytokines. The data on a condition of an immunological homeostasis which are available in domestic and foreign literature convincingly confirm permanent pathological shifts both in cellular, and in humoral immunity links. The analysis of the immune status showed that at most sick hypermarkets at normal quantity in a vascular bed of V-lymphocytes the average content of T-cells is considerably reduced in comparison with indicators of healthy donors. Decrease in both T-suppressor, and T-helper lymphocytes is noted, and the number of the last decreases more significantly. Many authors consider a relative lymphopenia as the most often found hematologic sign of hypermarket. Others, besides, note existence of an absolute lymphopenia in a tumoral stage of a disease. The functional lymphocyte activity at sick hypermarkets, according to a number of authors, is lowered. Others report about lack of reliable distinctions in the nature of proliferation of peripheral blood lymphocytes of ill and healthy faces. It is established that the course of pathological process at hypermarket is followed by decrease of the activity of natural killers.

Results of researches of a condition of a humoral link of immunity are contradictory. In a number of works essential disturbances in products are not revealed by V-lymphocytes of immunoglobulins. At the same time there are instructions on existence at hypermarket of explicit IGM-and the hidden IgA-and IgG-deficit of antibodyformation. Other researchers revealed increase in blood of sick hypermarkets of number of IgA, IGM, and also significant increase in concentration of IgG. At the same time increase in level of immunoglobulins is explained with a helper phenotype of tumoral substrate at hypermarket. Thus, changes of indicators of the immune status at sick hypermarkets are characterized by considerable variability and lack of the signs having unconditional diagnostic value.

Treatment of Fungoid mycosis:

There is an opinion that at the initial, favorably proceeding forms of fungoid mycosis it is reasonable to appoint conservative fortifying treatment (group B vitamins, vitamin C) and outwardly steroid ointments. Also sanatorium treatment is shown.

In more expressed stages of a disease it is more preferable to appoint a combination therapy: cytostatics, antineoplastic antibiotics. It is reasonable to combine Prospidinum with corticosteroids.

In these cases the effect occurs quicker at smaller doses of drugs in this connection the number of by-effects and complications decreases.

In a tumoral stage of fungoid mycosis apply a roentgenotherapy. Outwardly appoint the funds allocated for reduction of an itch, including corticosteroid ointments.