What is Skin Lymphocytoma (Caesare’s Disease)?
Lymphocytomas of the skin exist in several forms: lymphomatosis (lymphocytoma) of the skin such as Sesari disease, fungal mycosis, common B-cell lymphocytoma of the skin.
Sesari disease can be defined as lymphocytoma (lymphomatosis) of the skin (T-lymphocytic nature) with leukemization. Damage to the bone marrow, the release of lymphocytes in the blood, observed in Cesari’s disease, serve as the basis for its classification in some cases as chronic lymphocytic leukemia.
Symptoms of Skin Lymphocytoma (Caesary Disease)
This form causes progressive lymphatic infiltration of the skin, first manifested by erythema, itching, increased exfoliation of the epidermis, then saucer-like and finally tumor-like growths. Until complete damage, the tumor is often located only on the face, back and legs. Skin lesions may precede the leukemia process for several years, but may be detected simultaneously with bone marrow damage.
In a developed form, Cesari syndrome is very characteristic: generalized erythroderma with marked edema, especially in the ankles, diffuse alopecia, nail dystrophy, increasing infiltration of the skin, especially of the face. Subjectively marked excruciating itching.
In the blood, the lymphocytes in Sesari disease look like atypical cells: their nuclei are of medium and large sizes (10-14 µm), of irregular shape, sometimes bean-shaped with looped twisted chromatin, but more often jagged, split, with hollow impressions; basophilic cytoplasm.
According to the immunological characteristics of T-lymphocytes in Sesari’s disease are T-helper.
Diagnosis of Skin Lymphocytoma (Caesary Disease)
The diagnosis in the early stages of the process with limited skin lesion and normal blood composition is established by the results of a skin biopsy; lymphatic infiltration is diffusely located under the epidermis and is not associated with the vessels, often forming a continuous layer of cells in the upper layers of the dermis.
Changes in the blood can be determined from the very beginning of the process, and already against the background of a generalized tumor. Lymphatic leukocytosis can in some cases reach several tens of thousands in 1 μl. During this period, bone marrow is usually affected, although moderately, lymphocytes do not exceed 30%. The leukemization of a lymphocytoma such as Sesari’s disease often serves as a basis for its inclusion in the number of forms of chronic lymphocytic leukemia.
Lymphatic infiltration of the lymph nodes, the spleen is unstable and insignificant, and liver and kidney infiltration may occur, leading to death.
The patient is primarily disabling the skin lesion with constant painful itching. Radiation therapy has the best effect, but its use is difficult with diffuse lesions. A good effect of continuous intravenous administration of cytosar (48-72 h) with tumor T-lymphatic infiltration of the skin (Otitis) has been described.