Sézary syndrome is a T-cell lymphoma and manifests as swelling of the skin, itching, and scaling, among other symptoms. The exact circumstances of its development are as yet unclear, which complicates treatment and prevention.
What is Sézary syndrome?
Sézary (Baccaredda) syndrome belongs to the group of T-cell lymphomas. A lymphoma is an abnormal enlargement of the lymph nodes, which can mean both a swelling and a tumor. The syndrome received its name from the French dermatologist of the same name. Sézary syndrome is a cutaneous T-cell lymphoma that affects certain cells of the immune system. Most of those affected are over 50 years old. Men are more likely to develop Sézary syndrome than women.
Causes
The cause of Sézary syndrome is T cells. T cells are special cells of the immune system that belong to the white blood cells. In Sézary syndrome, the T-cells are altered and thus mistakenly trigger a defense reaction of the immune system, resulting mainly in various inflammatory symptoms. The nucleus of the monocytoid and lymphocytoid cells is large and appears folded. In addition, in Sézary syndrome, these cells are characterized by a narrow cytoplasmic space. In some circumstances, the cell has multiple vacuoles grouped around the nucleus that appear like a collar. The signs thus closely resemble the picture seen in another form of T-cell lymphoma, namely mycosis fungoides. Special tests can detect these abnormal cells in Sézary syndrome in the skin, bone marrow and blood. Medical professionals usually consult a blood and/or skin test for diagnosis.
Symptoms, complaints, and signs
The most noticeable symptom of Sézary syndrome is extensive redness of the skin, which physicians refer to as erythroderma. Erythroderma is an inflammatory reaction of the human body, which manifests itself superficially in the form of the reddened skin all over the body. As a rule, it causes affected tissues to lose salt, protein and fluid, which brings an increased risk of infection. In the case of Sézary syndrome, medicine also speaks of malignant reticulemic erythroderma. It can take the form of pyoderma, which is a purulent, burning inflammation that is another symptom of Sézary syndrome. Other signs of the disease include itching, scaling, or keratinization of the skin, which medicine refers to as hyperkeratosis. Hyperkeratosis makes the outermost layer of the skin, which is the striatum corneum, thicker as more keratinocytes or corneocytes are formed. Hair loss all over the body may also be a potential sign of Sézary syndrome. The condition can also cause semi-annular wrinkling. This causes the skin to droop in typical areas, such as the elbows or buttocks, because the tissues no longer hold it taut. As a result, a pattern develops that is remotely reminiscent of cellulite, but spreads more evenly across the skin. Typically, swelling of the lymph nodes is also apparent, and occasionally changes may appear on finger and toe nails.
Diagnosis and course of the disease
Elevated lymphocyte levels, in addition to skin symptoms, may provide an initial clinical clue. Examination of the blood and the cells it contains may provide clarity. In laboratory testing, the PAS reaction is crucial. The “Periodic acid-Schiff reaction” is a staining technique that makes carbohydrates visible under the microscope. The stained parts of the cell subsequently appear in the blue-violet color spectrum and can thus be more clearly distinguished from one another. Differentially, physicians must first and foremost rule out neurodermatitis, psoriasis, and mycosis fungoides. The latter is a disease that also belongs to the T-cell lymphomas and therefore has a very similar clinical picture. Mycosis fungoides is more common than Sézary syndrome and forms tissue nodules.
Complications
In Sézary syndrome, patients suffer from various skin complaints. In most cases, this also results in significantly reduced aesthetics, so that most patients are ashamed of the complaints and also do not feel comfortable with them. This can also lead to the development of depression and inferiority complexes.Patients suffer from itching and redness of the skin. The skin itself may flake or be affected by a rash. Due to permanent scratching, scars often form as well. Furthermore, Sézary syndrome in many cases leads to hair loss, which occurs on the entire body. Young people in particular can become victims of bullying or teasing and also suffer from psychological upsets. Sézary syndrome can also discolor or generally change the nails. In most cases, the symptoms can be treated well. Taking vitamin A also has a very positive effect on the disease and can limit the symptoms. Special complications do not occur. Furthermore, the life expectancy of the patient also remains unchanged by Sézary syndrome.
When should one go to the doctor?
The affected person is definitely dependent on medical treatment by a doctor for Sézary syndrome. There can be no self-healing, so treatment by a doctor must be given in any case. The sooner the doctor is consulted, the better the further course of this disease. Therefore, a doctor should be consulted already at the first complaints and symptoms of Sézary syndrome. The doctor should be consulted if the person suffers from redness of the skin, which appears without any particular reason and does not disappear on its own. It spreads all over the body and leads to a significant decrease in aesthetics. Furthermore, severe itching or the formation of dandruff may also indicate Sézary syndrome and should also be examined by a doctor. Most of the people affected by this disease also suffer from hair loss. As a rule, Sézary syndrome can be detected by a dermatologist or by a general practitioner. Further treatment depends on the exact symptoms. As a rule, this disease does not reduce the life expectancy of the affected person.
Treatment and therapy
The cure rate for Sézary syndrome is about 50 percent. A commonly used treatment method is photochemotherapy, which also goes by the name psoralen plus UV-A (PUVA). PUVA combines irradiation with UV-A light with the application of psoralen. Psoralen is a group of natural substances. They are found in essential oils and are of plant origin. The role of psoralen in PUVA is to sensitize the skin to light. For this purpose, patients take the psoralen orally or have it applied to the area of skin for which treatment is scheduled. Creams or water solutions containing the psoralen can be used for this purpose. Depending on which form of PUVA is used, the interval between psoralen administration and irradiation may vary. The aim of PUVA is to contain the spread of the abnormal cells and possibly even to drive them back completely. The same goal is pursued by treatment with retinoids. These are substances that are similar to vitamin A. Medicine uses retinoids as a means of chemotherapy and applies them mainly when Sézary’s syndrome is already in an advanced stage. Interferons may also be considered for treatment of the disease. They are thought to inhibit the growth of abnormal T cells, on the one hand, and to activate killer cells that occur naturally in the human body, on the other.
Prevention
Prevention of Sézary syndrome mainly involves general recommendations: Healthy diet, avoidance of potentially carcinogenic substances, and a health-promoting lifestyle. The exact conditions under which the disease develops have not yet been conclusively clarified; this makes targeted prevention difficult. However, recent findings provide evidence that iron in the diet may also be relevant for Sézary syndrome. Genetic causes are also under debate: Sézary syndrome sufferers appear to lack certain gene sequences. In addition, research has been able to find evidence of missing tumor suppressors that normally cause cell growth to stop.
Follow-up
Life-threatening Sézary syndrome requires comprehensive therapy followed by follow-up care. The latter aims to avoid the carcinogens whenever possible. That is why it is extremely important for patients to follow medical recommendations. After medical treatment, it is helpful to live consciously and eat a balanced diet.Although there is still no conclusive knowledge about the causes of the syndrome, a health-conscious diet is helpful as a preventive measure and aftercare. Those affected can also take advantage of genetic counseling. General aftercare measures can support the recovery process. These include sensible skin care, which alleviates the redness and itching caused by the disease. Changes to the nails can also be avoided or at least reduced with the help of a balanced diet. If weight loss, fever or other complications occur following medical therapy, immediate medical examination is necessary. Sufferers should watch out for such symptoms to improve their chances of recovery. For detailed advice, patients can turn not only to their doctor. Those affected can also obtain comprehensive information and recommendations on treatment and aftercare at the Max Delbrück Center in Berlin.
What you can do yourself
Sézary syndrome is primarily treated with medication. The patient is prescribed MTX, prednisone, and similar agents that relieve symptoms and improve the appearance of the skin. Various general measures support the recovery process. Good skin care alleviates itching and redness. Nail changes can be reduced by a healthy diet. Lymphatic symptoms must be treated by a doctor in any case, as various complications such as weight loss or fever may occur. Sézary syndrome is a life-threatening disease with poor overall prospects for cure. This makes it all the more important to have a comprehensive consultation with a specialist who can show ways and means to improve the quality of life and improve the prognosis. Together with the doctor, measures such as chemotherapy or surgery should be discussed, since the prospects of cure are better the earlier therapy is started. The most important self-help measure is to determine the medical options and to make full use of them with the support of a specialist. The Max Delbrück Center for Molecular Medicine in Berlin-Buch is considered one of the most important institutes for Sézary syndrome research. Sufferers turn to the center for the best possible advice and treatment.
