Skin lymphoma is a tumor lesion of the skin resulting from the malignant proliferation of lymphocytes in it. Depending on the type of multiplying lymphocytes, T- and B-cell lymphomas are distinguished. The disease is manifested by the formation of nodules, plaques or erythrodermic areas on the skin, which is accompanied by an increase in lymph nodes. Diagnosis is carried out by histological examination of biopsy material from the affected area. In the treatment of skin lymphoma, chemotherapy, radiation therapy, PUVA therapy, extracorporeal photophoresis are used.
General information
According to studies, T-cell skin lymphomas occur in 65-70% of cases, while B-cell skin lymphomas account for 20-25%. Another 10% is occupied by so-called unclassifiable skin lymphomas.
Causes
The development of skin lymphoma is associated with a mutation of T or B lymphocytes, which leads to their uncontrolled reproduction and migration into the skin. The exact reasons triggering this mechanism are not known. It is assumed that the occurrence of a malignant clone of lymphocytes can be provoked by constant antigenic stimulation against the background of impaired immune protection of the body.
The provoking role is assigned to viral infections caused by retroviruses, cytomegalovirus, herpes simplex virus type 8, Epstein—Barr virus. The action of various chemicals and carcinogens used in agriculture, chemical industry, construction and other fields can also be the cause of skin lymphoma.
Skin lymphoma is primary when the disease begins with a lesion of the dermis, and secondary — as a result of the migration of lymphocytes from the lymphoid organ in which their reproduction occurs. Such organs include the bone marrow, thymus gland, lymph nodes, spleen, lymphoid clusters along the respiratory tract and gastrointestinal tract.
Skin lymphoma symptoms
Skin lymphomas are characterized by polymorphism of the rash (spots, plaques, nodes), varying degrees of severity of itching and an increase in peripheral lymph nodes. According to the degree of malignancy, lymphomas of I, II and III degrees are distinguished. According to clinical manifestations: nodular, plaque and erythrodermic forms. The nodular form of grade I skin T-cell lymphoma is characterized by small flat nodules the size of a millet grain. The nodules have a lilac or yellowish color, are arranged in groups and are prone to spontaneous regression. With a more malignant course, the nodules increase, acquire a cherry color and lose the tendency to grouping. Patients die from metastases after 2-5 years.
A small-nodular form of T-cell lymphoma of the skin is rare, in which follicular nodules merge into plaques with a surface peeling resembling psoriasis. Against this background, large nodules appear, which then undergo necrosis. The plaque form of grade I skin T-cell lymphoma is represented by indistinctly delimited yellowish plaques. The size of the plaques may be larger than the palm of your hand. They gradually resolve with the formation of areas of atrophy and hyperpigmentation.
The plaque form of the II degree (mushroom-like mycosis Alibera) occurs in 26% of all skin lymphomas. It is characterized by stage-by-stage development. Initially, flaky bright pink spots and other elements appear (erythematous stage). Then stagnant-red plaques form on the spot, often with a wet surface and peripheral growth (plaque stage). In the tumor stage, plaques are replaced by flat nodes up to the size of an orange with necrosis in the center of the formation.
The erythrodermic form of grade I skin T-cell lymphoma (pre-Cesari syndrome) often develops against the background of long-term, for 10-15 years, existing eczema or neurodermatitis. The skin is reddened and edematous, covered with large plates of white scales. There is a generalized increase in lymph nodes, nail dystrophy, hair loss, fever and excruciating itching. After a few years, the patient may die from cachexia or the process turns into a grade II erythrodermic form (Cesari syndrome), characterized by pronounced infiltration, peeling and dry skin.
B-cell lymphomas of the skin are characterized by the absence of itching and other subjective sensations with grade I and II malignancy. They are manifested by a plaque and nodular shape. The plaque form is characterized by the same stages as for T-cell lymphoma of the skin. The nodular shape develops with the formation of one or more hemispherical nodes of a tightly elastic consistency, the size of which reaches the size of a walnut.
Diagnosis
In many cases, skin lymphomas are accompanied by changes in the clinical blood test. T-cell lymphoma is characterized by leukopenia and monocytosis. In pre-Cesari syndrome, leukocytosis and neutrophilosis are observed, an increase in the number of eosinophils. With Cesari syndrome, there may be an increase in white blood cells up to 30000-2001. B-cell lymphomas of the skin are characterized by the appearance of normochromic at first, and then hemolytic anemia.
Histological and cytological examination of the material taken by biopsy of elements of skin lymphoma and, if necessary, lymph nodes is of crucial diagnostic importance. A biopsy makes it possible to differentiate T and B cell lymphoma of the skin, as well as to determine the degree of its malignancy. When internal organs are involved in the process, they are examined: ultrasound of the abdominal cavity, lungs x-ray, CT of the lungs, etc.
Treatment and prognosis
The main method of treating patients with skin lymphoma is chemotherapy. It uses cytostatics (vincristine, vinblastine, cyclophosphane), corticosteroids (prednisone, betamethasone) and interferons (gamma interferon). In the treatment of individual spots, plaques and single tumors, radiation therapy, PUVA therapy, phototherapy are used. In some cases, extracorporeal photophoresis is effective. They often combine various methods of treatment and the drugs used. For example, radiation is prescribed together with chemotherapy and after it.
With timely initiation of treatment and grade I-II malignancy of skin lymphoma, it is often possible to achieve pronounced remission and prolong the patient’s life. In this case, intercurrent diseases or complications of treatment lead to a fatal outcome. If skin lymphoma is diagnosed in the tumor stage or has a pronounced malignancy, the prognosis is extremely unfavorable, a fatal outcome may occur 2 years after the onset of the disease.
