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Skin lymphoma — clinically and morphologically heterogeneous group of the tumors developing owing to malignant proliferation of lymphocytes in skin.
Epidemiological and statistical data on malignant lymphoma of skin in Russia are absent, however practice demonstrates increase in number of such patients. According to national institute of cancer of the USA, for the last 20 years the number of patients with the T-cellular lymphoma (hypermarket) doubled, having reached 100 thousand cases a year. Most often (in 65% of cases) T-lymphoma meet, V-cellular tumors make 25% which are not classified — 10% of all ZLK.
Classification The standard classification does not exist. The majority of classifications of tumors of an adenoid tissue is based on their morphological and immunological features. Malignant lymphoma of skin subdivide:
• on primary (T-cellular, V-cellular, not classified);
Primary malignant lymphoma of skin on clinical signs divide also on stages (the I—III Art.) and zlokachestvennost degrees (low, high).
Etiology and pathogeny The etiology of malignant lymphoma of skin is not established. As a possible factor of development of malignant lymphoma of skin retroviruses, a virus of the T-cellular leukosis of the person (HTVL-1), a cytomegalovirus, Epstein's virus — Burra, a herpes simplex virus 8 types are considered; the chemicals used in the industry, construction, agriculture; the raised radiation and insolation; reception of some HP. These factors increase risk of emergence of a mutant clone of lymphocytes and strengthen their migration in skin. At a constant antigen challenge and disturbances in an immunological defense system conditions under which the arisen malignant clone of lymphocytes can survive and increase are created.
Points detection at separate nosological forms of malignant lymphoma of skin of certain histocompatibility antigens of the HLA system of a class I to a role of hereditary factors. Histocompatibility antigens of V-5 and V-35 often occur at patients with malignant lymphoma of skin of high degree of a zlokachestvennost, and A-10 antigens — at patients with malignant lymphoma are skin of less aggressive current.
Tumoral lymphocytes can be formed in any lymphoid body (a thymus, marrow, a spleen, lymph nodes, lymphoid accumulations on the course of a digestive tract and respiratory tracts) and to disseminate in skin (secondary malignant lymphoma of skin). At primary malignant lymphoma of skin primary defeat of a derma at the initial stages of pathological process takes place.
The big part in emergence of malignant lymphoma of skin is assigned to skin as the body which is actively participating in generation of local and general immune responses with involvement of all immune system. The considerable part of limfoproliferativny diseases is considered as a tumor of actually immune system of skin.
Clinical signs and symptoms Are characteristic of malignant lymphoma of skin:
• polymorphic rashes;
• itch of different degree of manifestation;
• increase in regional lymph nodes;
• change of a blood count, involvement in pathological process of internals (at late stages).
The T-cellular lymphoma (hypermarket, Cesaria's syndrome) is characterized by staging of tumoral process. In an initial stage of rash have nonspecific character, can remind a banal dermatosis (seborrheal eczema, pink deprive, atopic dermatitis, a blyashechny parapsoriasis, red flat deprive). Often there is a palmar and bottom hyperkeratosis with deep cracks. Spotty elements infiltrirutsya, become infiltrative blyashechnymi (a blyashechny stage). Blyashkovidny elements expand, turn in tumoral, tumors develop on the unimpaired skin. Gradually they ulcerate, break up.
Despite possibility at patients with malignant lymphoma of skin of damage of internals (lungs, a liver, etc.), the disease is long is limited to skin, bodies of a hemopoiesis are not involved in pathological process or suffer only in an end-stage.
Except a classical form of hypermarket is available erythrodermic which most of authors identifies with Cesaria's syndrome. The last is characterized by a fever, an indisposition, a polyadenitis, increase in a liver, spleen, changes of clinical blood test (a lymphocytosis, emergence of cells of Cesaria — patholologically the changed lymphocytes with tserebriformny kernels).
The long, chronic course, absence of an itch is characteristic of a poykilodermichesky form of T-cellular malignant lymphoma of skin.
At V-cellular lymphoma of rash of a monomorfna, there is no staging of process, there is no itch. Without the previous stages on normal skin tumors develop. They are brilliant, red, with a lividny shade, a myagkoelastichesky consistence. Sometimes rashes have an appearance of arches, semi-arches, disks. Elements increase in sizes and in number, quickly ulcerate.
The diagnosis and the recommended clinical trials Stages of diagnosis of a lymphoma of skin:
The 1st stage — physical inspection;
The 2nd stage — laboratory and tool diagnosis. Pathomorphologic, immunomorphological diagnosis, tool research of a condition of internals is conducted.
Obligatory methods of research:
• clinical and biochemical analysis of blood;
• research of the immune status;
• Ultrasonography of abdominal organs and retroperitoneal space;
• X-ray analysis of bodies of a thorax;
• computer tomography of bodies of an abdominal, chest cavity, small pelvis;
• sternal puncture. Additional methods of research:
• a biopsy of the increased lymph nodes with immunophenotyping.
Differential diagnosis The differential diagnosis is carried out between malignant lymphoma of skin and atypical forms of a high-quality dermatosis, other tumors of skin (not malignant lymphoma of skin), benign limfoproliferativny diseases of skin, secondary damage of skin at malignant diseases of blood.