What is CREST syndrome? What are the signs of the disease? How is the treatment of the disease? What is prevention? You can get answers to these and other questions by reading our article.
General information
Definition CREST syndrome is a designation for a disease known as a limited variant of scleroderma. A feature of the disease is the appearance in the structure of the connective tissue of the affected areas in the form of peculiar plaques.
The mechanism for the development of pathology has not yet been studied. Doctors believe that the "starting point" are violations that occur in the endocrine system. In particular, the development of characteristic symptoms is caused by excessive secretion of serotonin. Against the background of the process, vasospasms occur, as well as a change in the composition of biological lubricants. The immune system begins to perceive such failures as an attack on the body of foreign cells. Under the influence of lymphocytes and leukocytes in healthy tissues, inflammatory processes begin to occur. However, activation of blood cells, which play a protective role in the body, does not lead to positive results. The body again starts similar processes, which causes the formation of new foci of tissue inflammation.
Limited scleroderma: signs of the disease
People who are developing pathology may complain of a feeling of tightening of the skin, soreness of tissues in various parts of the body, as well as itching and tingling. Signs of the development of the disease are the appearance of puffiness, atrophy and seals on the surface of the epidermis.
Typically, CREST syndrome is manifested by the formation of lilac and pinkish spots on the skin of a rounded shape. The sizes of such plaques can vary from the size of the coin to the child's palm. Most often, such manifestations are concentrated in the region of the upper and lower extremities, nearby mammary glands, on the face and body. In the affected areas, the skin is difficult to fold. There is a lack of sweat separation, hair growth stops.
Subsequently, such a symptom of the disease, scleroderma as skin tightening, practically disappears. At the same time, pigmented areas appear in the tissue structure. If proper attention is not paid to the treatment of the disease, muscle tissues, bones are damaged, in rare cases, internal organs.
Prerequisites for the development of the disease
The specific causes of the formation of the pathology characteristic of the vast majority of clinical cases remain a mystery to the researchers of the problem. It is only known for certain that such factors can provoke the development of a CREST syndrome:
- Disorders in the functioning of the endocrine system;
- Hypothermia, overheating and mechanical damage to the skin;
- Ingestion of foreign proteins during blood transfusion or vaccination;
- Regular exposure to humans of substances in organic solvents, ingestion of silicon dust in the lungs;
- Uncontrolled, unauthorized use of certain pharmacological preparations.
Drug treatment
The treatment of CREST syndrome involves a combination of drugs whose active substances stimulate the immune system, have an antifibrotic effect and contribute to an improvement in the circulation of body fluids. The treatment program is individually. The choice of medications depends on the nature of the course of the disease, its severity and degree of development.
With CREST syndrome, patients are most often prescribed the following pharmacological preparations:
- Prednisone - is used in cases where there is a significant spread of foci of scleroderma in various parts of the body. In order to eliminate the characteristic symptoms, the medicine is taken at 20-30 milligrams per day. If improvements are observed, the dose is progressively reduced until the drug is completely abandoned.
- Sodium salt - is applied topically and makes it possible to stop foci of tissue damage. The tool is effective only in the initial stages of the development of the disease.
- Hyaluronidase - looks like an effective solution in the fight against the occurrence of seals in the structure of the skin. The substance is used internally and externally.
- "Diabazole" - a drug containing polyenzymes. It makes it possible to slow down the development of a disease that does not yet have significant progress.
- "Koplamin" - the drug has a positive effect on the state of blood vessels.
Physiotherapy
Physiotherapeutic procedures are also quite effective in treating limited scleroderma. The main advantage of such decisions is the possibility of a targeted effect directly on the lesion foci of tissue.
Therapy based on physiotherapy involves the use of the following methods:
- Electrophoresis
- The impact on the tissue of diadynamic currents.
- Ultrasound.
- Reception of mud baths.
- Laser therapy of low frequency.
- Magnetic therapy.
- Chemotherapy (in severe cases of the disease).
Prevention
For people who are diagnosed with "limited scleroderma," doctors recommend avoiding exposure to the skin in direct sunlight. In addition, such persons need to prevent overheating and hypothermia of the body, mechanical injuries, stress.
The best prevention of CREST syndrome is to be under the supervision of a physician. This is the only way to track the progress or retreat of the disease, and also prevent the development of concomitant connective tissue pathologies against the background of the problem. The patient requires regular visits to the office of a dermatologist, venereologist, endocrinologist and neuropathologist.
Chances of recovery
In the case of the development of CREST syndrome, the prognosis is quite favorable. A strict adherence to the doctor’s recommendations, a combination of medical and physiotherapeutic treatment - all this in most cases makes it possible to avoid the appearance of new foci of scarring of tissues.
Unfortunately, skin areas that have already undergone pathological changes remain so for the rest of their lives. It is quite difficult to completely avoid the appearance of cosmetic defects with a disappointing diagnosis. The reason for this is very vague symptoms and the causes of the formation of the disease, which prevents the timely detection of the disease. Be that as it may, limited scleroderma does not pose a threat to life.