Cesari syndrome is a malignant skin lesion characterized by tumor transformation of T-lymphocytes. Cesari syndrome is manifested by the following typical clinical signs:
lymphadenopathy;
erythroderma;
the presence of specific cells in the blood (usually such cells have folded nuclei).
The content of the article:
Symptoms of Cesari syndrome
Diagnosis of Cesari syndrome
Treatment of Cesari syndrome
Cesari syndrome
The diagnosis of Cesari syndrome is made on the basis of the clinical picture, taking into account the blood test data for the presence of Cesari cells in it, as well as the results of a skin biopsy. To identify how much the malignant process has spread in the body, an examination of the internal organs is carried out. The methods of treatment of Cesari syndrome include chemotherapy, radiation exposure, photodynamic therapy, immunotherapy, as well as treatment with retinoids.
The first information about the Cesare syndrome was first published by French scientists back in 1938. Even then, scientists named Sezari and Bovrain identified three main signs of this disease. Over time, the disease and the cells that cause it were named after the author who first described them.
In modern dermatology, Cesari syndrome belongs to the group of T-cell lymphomas of the skin. Together with the so-called fungal mycosis, Cesari syndrome is considered one of the most common diseases of this group. It should be said that most often Cesari syndrome is observed in elderly men. But it can also manifest itself in people of other ages and other genders.
Symptoms of Cesari syndrome
At the beginning, the Cesari syndrome occurs in two variants.
Pre-erythrodemic period
The first variant is characterized by a pre-erythrodemic period, which is manifested by scattered rashes on the skin in the form of erythematous spots (or plaques). From the moment of occurrence of such plaques to the development of total erythroderma, it takes from several weeks to several years.
Most often, there is a rapid onset of this disease, in which erythematous-infiltrative substances merge with their further rapid development of erythroderma. It should be said that the pre-erythrodermic period is almost not allocated.
Erythrodermic stage
Regarding the erythrodermic stage of Cesari syndrome, it should be noted that its clinical picture is very similar to the clinic of the erythrodermic variant of fungal mycosis. The affected skin has a bluish or bright red color. Infiltration leads to thickened skin, which looks rough and is very difficult to fold. A distinctive feature of this stage of Cesari syndrome from fungal mycosis is considered to be a significant severity of skin dyschromias, which includes:
onychodystrophy;
alopecia;
depigmentation;
hyperpigmentation;
hyperkeratosis of the palms and feet;
ectropion;
poikiloderma.
Patients with Cesari syndrome have lymphadenopathy, a significant increase in axillary, inguinal, femoral and cubital lymph nodes. During probing, such nodes are characterized by an elastic surface, painless and dense tissues, which are also not soldered to other nearby surfaces. Approximately 30% of people have an enlarged spleen and liver.
With Cesari syndrome, lymphadenopathy and erythroderma occur with pronounced disorders of the patient’s well-being. Patients are characterized by weakness, impaired sweating, elevated body temperature (up to 39 °) and chills. Patients may complain of severe itching, colic and burning on the surface of the skin.
Regarding the spread of the malignant process itself, there are four stages of Cesari syndrome.
The first stage
The first stage includes: erythematous spots (as well as plaques), which cover less than 10% of the entire skin surface (IA); erythematous spots, plaques, spread only over 10% of the entire skin.
The second stage
The second stage includes erythroderma, spread over a significant area of the skin (IIA); erythroderma, combined with the appearance of one or more tumors on the dermis, while the lymph nodes are enlarged, and there are no signs of metastasis (IIB).
The third stage
In the third stage, erythroderma is spread over the entire surface of the skin, usually accompanied by the presence of plaques, tumors and lymphadenopathy. Interestingly, metastasis is not detected at this stage.
The fourth stage
The fourth stage includes the following types of the course of the disease:
when erythroderma, tumors, plaques can be detected on most of the entire skin surface, metastases located in the lymph nodes (IVA) are usually found against the background;
a significant part of the skin is affected, enlarged lymph nodes are observed in which metastases can be detected;
the metastases themselves are also visible in the internal organs of the patient.
Diagnosis of Cesari syndrome
To identify Cesari syndrome, it is enough for a dermatologist to detect the following clinical picture: combined lymphadenopathy and erythroderma. The detection of Cesari cells in the blood test taken will help confirm the diagnosis. For this purpose, immunophenotyping of the sample taken, which is obtained during a skin biopsy, is also used. To determine the stage of Cesari syndrome, as well as the degree of prevalence of its cells throughout the body, only an additional examination of the patient will allow. The survey will include:
chest x-ray;
CT of the kidneys;
MRI and MSCT;
Ultrasound of the peritoneal organs;
a biopsy of the lymph nodes.
Differential diagnosis of Cesari syndrome is usually carried out with various benign diseases that are accompanied by erythroderma (psoriasis, atopic dermatitis, true eczema, lupus erythematosus. In the case of these dermatoses, erythroderma is characterized by a secondary character. The latter can be identified with a thorough interview of the patient and his anamnesis data. It is also necessary to differentiate the Cesari syndrome, for example, from fungal mycosis, leukemia and systemic lymphomas.
Treatment of Cesari syndrome
In the treatment of Cesari syndrome, the use of photodynamic therapy, radiation and immunological methods, chemotherapy, as well as retinoid therapy is indicated.
Interestingly, photodynamic therapy consists in the fact that a special drug is injected intravenously into the patient. Such a drug, as a rule, accumulates in tumor cells. After that, the patient’s skin is irradiated, or the practice of extracorporeal blood irradiation is permissible, activating the injected substance. The result of activation will be the destruction of malignant cells.
If we talk about the use of chemotherapeutic drugs in systemic therapy, they are usually prescribed orally or used as injections. Regarding the stage of Cesari syndrome, topical, regional chemotherapy is carried out, applying drugs to the skin, as well as injecting them into specific affected areas in the patient’s body.
Radiation therapy is carried out through external influence, the introduction of radioactive medications inside. In the case of Cesari syndrome, electron exposure is applied throughout the skin. If we talk about the immunotherapy of Cesari syndrome, it is carried out using alpha-interferon, monoclonal antibodies and interleukin-2. Retinoid treatment is based on the ability to stop the growth of malignant cells.
