Sezary syndrome is a malignant skin lesion caused by tumor transformation of T—lymphocytes and manifested by a triad of typical clinical signs: erythroderma, lymphadenopathy and the presence of specific cells in the blood with folded nuclei. The diagnosis is established on the basis of a characteristic clinical picture, taking into account the data of a blood test for Sezary cells and the results of a skin biopsy. To determine the prevalence of the malignant process, an examination of the internal organs is carried out. The methods of treatment include: chemotherapy, photodynamic therapy, radiation exposure, immunotherapy, retinoid treatment.
Information about the Sezary syndrome was first published by French scientists Sezari and Bovrain in 1938. They also identified three main signs of the disease. Over time, both the syndrome and its specific cells with folded nuclei were named after the author who first described them.
Modern dermatology classifies Sezary syndrome as a group of T-cell lymphomas of the skin. Along with fungal mycosis, it is one of the most common diseases of this group. In elderly men, Sezary syndrome is observed somewhat more often than in other categories of the population. However, it can develop in people of both sexes and at any age.
The onset can occur in two ways. In the first variant, there is a pre-erythrodermic period, manifested by the appearance of scattered rashes on the skin in the form of erythematous spots or plaques. From the moment of their occurrence to the development of total erythroderma, it can take from several weeks to 2-3 years. However, more often there is a rapid onset of Sezary syndrome, in which there is an active fusion of erythematous-infiltrative elements with the rapid development of erythroderma. At the same time, the pre-erythrodermic period is practically not allocated.
The erythrodermic stage of Sezary syndrome is similar to the clinical picture of the erythrodermic variant of fungal mycosis. The affected skin has a stagnant bluish or bright red color. Pronounced infiltration leads to thickening of the skin, it looks rough and hardly gathers into folds. Unlike fungal mycosis, skin dyschromias are more pronounced in Sezary syndrome: onychodystrophy, depigmentation and hyperpigmentation, alopecia, hyperkeratosis of the feet and palms, ectropion, poikiloderma.
All patients with Sezary syndrome have lymphadenopathy. There is an increase in axillary, femoral, inguinal, cubital lymph nodes. When probed, they have an elastic consistency, dense and painless, not soldered to the surrounding tissues. In 25-30% of patients, an enlargement of the spleen and liver is detected.
Erythroderma and lymphadenopathy in Sezary syndrome are accompanied by a pronounced violation of the patient’s condition. Characterized by general weakness, sweating disorders, body temperature increases up to 39 °, accompanied by severe chills. Patients complain of intense itching, tingling and burning sensation of the skin.
Depending on the prevalence of the malignant process, there are 4 stages of Sezary syndrome.
- IA — erythematous spots and plaques capture less than 10% of the skin surface
- IV — erythematous spots and plaques are spread over 10% of the skin and more
- IIA — erythroderma is spread over a large area of the skin; there is an increase in lymph nodes, but their biopsy does not reveal metastases
- IIB — erythroderma is combined with the formation of one or more tumors on the skin; lymph nodes are enlarged, without signs of metastasis
Stage III — erythroderma is spread over the entire surface of the skin, accompanied by the presence of plaques and tumors, lymphadenopathy; metastasis to lymph nodes is not detected.
- IVA — erythroderma, plaques and tumors are detected on most of the skin; against the background of lymphadenopathy, metastases to the lymph nodes are detected
- IVB — most of the skin is affected; lymph nodes are enlarged, metastases are detected in them; metastases are also detected in internal organs.
The dermatologist can suspect Sezary syndrome due to the characteristic clinical picture of the disease — a combination of lymphadenopathy and erythroderma. The detection of Sezary cells in the patient’s blood test confirms the diagnosis. For the same purpose, immunophenotyping of a sample obtained during a skin biopsy is used. Determining the stage of the disease and the extent of the spread of tumor cells throughout the body requires additional examination of the patient. The latter includes chest X-ray, MRI, CT of the kidneys, MSCT or ultrasound of the abdominal cavity and pelvic organs, biopsy of lymph nodes.
Differential diagnosis of Sezary syndrome is performed with benign dermatological diseases accompanied by erythroderma: atopic dermatitis, psoriasis, true eczema, systemic lupus erythematosus, etc. With these dermatoses, erythroderma is, as a rule, secondary in nature and a thorough interview of the patient reveals in his anamnesis data on a previous disease. It is also necessary to differentiate Sezary syndrome from fungal mycosis, systemic lymphomas and leukemias.
In the treatment of Sezary syndrome, photodynamic therapy, chemotherapy, radiation and immunological methods, retinoid therapy can be used.
Photodynamic therapy consists in intravenous administration of a special drug to the patient, which accumulates mainly by tumor cells. Then the patient’s skin is irradiated or extracorporeal irradiation of his blood is performed, which activates the injected substance. As a result of activation, the drug begins to destroy malignant cells.
Chemotherapeutic drugs for systemic therapy are prescribed orally or in injections. Depending on the stage of Sezary syndrome, topical or regional chemotherapy can be performed with the application of drugs to the skin or their introduction into individual affected areas of the body.
Radiation therapy can be carried out both by external influence and by the introduction of radioactive drugs inside. With Sezary syndrome, an electron beam is also applied to the entire skin. Immunochemotherapy of Sezary syndrome is performed using alpha-interferon, interleukin-2 or monoclonal antibodies. Retinoid therapy is based on their ability to inhibit the growth of malignant cells.