Scleroderma
Scleroderma is a diffuse disease in which scar tissue begins to be deposited in the entire skin or in some parts of it. With the progression of the disease, scar tissue appears not only on the skin, but can occur in internal organs: in the lungs, in the musculoskeletal system, digestive tract, in the heart and even in the kidneys.
The content of the article:
Causes of scleroderma
Symptoms of scleroderma
Stages and types of scleroderma
Diagnosis of scleroderma
Treatment of scleroderma
Scleroderma
Causes of scleroderma
What exactly are the causes of scleroderma — no one knows for sure. However, the disease has been known for a long time and is common in many countries, in all ethnic groups, starting with infants and ending with the elderly. According to statistics, scleroderma is four times more often diagnosed in women than in men, and the incidence of the disease increases significantly over the years. Some scientists believe that scleroderma may have a genetic predisposition. The exact mechanism of the disease is unknown. In some cases, this disease may occur after the patient has suffered acute or chronic infections (diphtheria, scarlet fever, erysipelas, tuberculosis, and others).
Symptoms of scleroderma
Pathology mostly develops in the vascular wall and connective tissue structures. Due to the action of sclerotin, vascular spasm occurs. The connections between the hyaluronidase enzyme and hyaluronic acid are also disrupted, which in the future leads to a significant accumulation of mucopolysaccharides. Fibrinoid degeneration also occurs in connective tissues and, as a result, collagen synthesis increases. The pathological sclerodermic process quickly spreads to the nerve endings and even to all branches of blood vessels. A confirmatory factor of scleroderma is considered to be an increase in serum titers of auto-antibodies to their own tissues, and an instant increase in gamma globulins is also detected.
There are 3 forms of scleroderma: limited, systemic and mixed.
Disorders in the endocrine system, hypothermia, trauma, neurotic disorders, genetic factors have a strong influence on the development of scleroderma. There are cases when scleroderma from the focal form passed into the systemic and affected the internal organs.
Limited scleroderma is characterized by the appearance on the skin of one or more plaques the size of a coin. Plaques on the skin have a sharply limited shape: they are dense, quite smooth and shiny, sometimes they can rise above the skin or sink a little. The color of the plaques is yellowish-grayish, with a characteristic lilac rim. They are oval, rounded and linear in shape. They are usually localized on the patient’s torso, face or limbs.
Stages and types of scleroderma
This type of scleroderma has 3 stages:
In the first, round bluish-red plaques are formed. At the same time, the patient’s skin is edematous, with a kind of dough consistency.
The second stage begins to develop after a couple of weeks. At this stage, hardening occurs. The skin becomes waxy, fits very tightly to the tissues, the pattern on the skin is smoothed, the hair cover on the affected area is completely absent, the skin in the focus of the disease cannot be assembled into a single fold.
At the third stage of the disease, the patient’s skin becomes thinner, becomes parchment-like in appearance. This is the stage of atrophy.
Also, in limited scleroderma, several subspecies are distinguished. Linear scleroderma is one of them. It usually appears on the face, directly on the skin of the frontal part and the scalp. This type of scleroderma looks like a trace of a saber strike. In some cases, the patient can observe linear scleroderma in the thoracic region, as well as on the lower extremities. This location of the focus of the disease indicates the neurotrophic pathogenesis of the disease. This form of the disease is often found in children.
Scleroderma can also be expressed in the form of small, rounded, rather smooth, sharply outlined, shiny spots. The spots have a porcelain-white coloring and a dense consistency. Most often, white spots are placed on the neck, shoulders, genitals, upper chest, in the oral cavity. This form of scleroderma is differentiated with lichen planus and leukoplakia.
Superficial scleroderma is another subspecies of a limited form of the disease, similar to plaques of bluish or brown color. The center of the plaques is a little sunken, vessels are visible in it. The patient with superficial scleroderma does not feel strong anxiety. Usually plaques can be found on the back and skin of the lower extremities.
Systemic scleroderma is a serious disease that in most cases begins in early or adolescence, girls are more likely to get sick with it. At the very beginning, the disease will affect the hands and face of the patient, and then the disease passes to the skin of the trunk and the skin of the limbs. The foci of the disease gradually increase in size and begin to harden. The affected skin is fixed to the underlying tissues, noticeably changes its color and becomes yellowish. Sometimes there may be vasodilation. The skin is very tense, the mobility of facial muscles is limited. The inguinal and axillary lymph nodes are enlarged. The initial symptoms of the disease are insomnia, a feeling of numbness of the limbs, a strong tightening of the skin. Then there is weakness, severe headache, joint and muscle pain, general malaise, and fever.
Diagnosis of scleroderma
The first stage of the plaque form of the disease is difficult to diagnose. It is also difficult to make an accurate diagnosis with diffuse scleroderma, since the symptoms of the disease in the initial stage are very similar to Raynaud’s disease. Only close observation of the patient allows the doctor to make the correct diagnosis.
There are no exact diagnostic signs of the disease. Therefore, if there are some prerequisites to believe that the swelling of the affected tissues is caused by scleroderma, it is necessary to exclude all pathologies in order to make the correct diagnosis.
The main signs of scleroderma are scleroderma skin lesion, musculoskeletal syndrome, positive Raynaud’s syndrome, fibrous and sclerotic changes in internal organs. Also, additional criteria are hyperpigmentation of the skin, telangiectasia, rapid settling of red blood cells in the blood and sharp weight loss. First of all, the doctor conducts a biochemical blood test, and if hypergammaglobulinemia is detected in it, there are antibodies to DNA and ANF and there is a positive reaction to rheumatoid factor, then the disease in the body is progressing.
Treatment of scleroderma
Simple forms of scleroderma usually go away on their own, without medical intervention. Systemic scleroderma requires long-term intensive therapy. The main principle of treatment of scleroderma is the appointment of antifibrotic drugs, such as Kuprenil and Duiciphon. The drugs should be taken according to the scheme prescribed by the doctor for 3-5 years. Only in this case it is possible to prevent the defeat of the disease of internal organs.
As an auxiliary therapy, enzymatic drugs and glucocorticosteroids are prescribed. Therapeutic massage, electrophoresis and laser therapy may be indicated locally. Acupuncture, carbon dioxide and hydrogen sulfide baths are recommended for all patients with scleroderma. A new technique in the treatment of scleroderma is stem cell therapy. When stem cells are introduced into the human body, protective properties increase, the body’s ability to form new vessels is activated. Stem cells can replace dead cells and thereby stop attacks of vascular spasm. Gradually, in the course of treatment, all fibrous changes in the internal organs are restored.
In order to prevent the occurrence or development of the disease, people who are genetically predisposed to this disease need to follow certain rules. The patient should protect his hands, ears, face from cold and excessive conditioning. Nutrition should be in the form of a balanced diet, complete abstinence from alcohol and smoking, strict maintenance of optimal weight; the patient should avoid overwork and stressful situations.
The outcome of the disease is very difficult to predict. In case of complete rejection of therapy and drug treatment, a fatal outcome is possible.
