Scleroderma is a disease of connective tissue, the main manifestations of which are skin sclerosis (condensation) and narrowing of small vessels. With scleroderma, the amount of collagen in the dermis increases. The incidence is the same in all age groups, regardless of gender. Exact diagnostic criteria for scleroderma have not yet been determined, so the exclusion of similar dermatological diseases is important in its diagnosis. Scleroderma therapy includes the appointment of antifibrotic agents, corticosteroids and enzyme preparations, laser therapy and physiotherapy procedures.
The etiology of the disease is not fully understood, but the presence of endocrine diseases in patients with scleroderma is much higher. Acceptance of certain drugs, transfusion of blood components and vaccination in a few cases provoke the development of scleroderma. Frequent hypothermia and genetic predisposition also increase the risk of getting sick.
The primary manifestations of scleroderma are edema and densification of the skin. This swelling is so tight that when pressing on the skin there is no depressions. The process begins with fingers and hands and progresses to the skin of the forearms, face. The trunk and lower limbs are involved in the process only in isolated cases. Limited scleroderma has a benign course and at the first stage is manifested only by swelling of the skin, then consolidation and skin sclerosis is added. With a prolonged course, tissue atrophy and pigmentation change occur. In children, linear scleroderma is more common. It is characterized by the appearance of areas of thickened skin in the form of long strips on the upper and lower limbs, less often on the face and on the scalp. Linear scleroderma basically ends in recovery. Only 5% of cases develop systemic scleroderma.
Plaque scleroderma also has a benign course. Clinically manifested yellowish waxy spots on the skin, which are surrounded by a pink or purple rim. Elements gradually become denser and become like plaques. Plaque scleroderma rarely progresses and usually ends on its own.
Systemic scleroderma is characterized by damage to the skin, musculoskeletal system and internal organs, which have connective tissue (heart, lungs, kidneys, digestive tract). Since scleroderma is a narrowing of the vessels, the vascular disorders proceed according to the type of Raynaud's syndrome, when circulatory disturbances are symmetrical and lead to necrosis of the tissues of the upper extremities. The fingers become pasty, and the skin acquires a cyanotic color. In women, systemic scleroderma, with fibrotic changes in the internal organs, is diagnosed seven times more often than in men.
The main cause that leads to systemic scleroderma is a disruption in the synthesis of connective tissue fibers, when the amount of collagen is significantly increased. Disturbances of microcirculation and immunodeficiency increase the likelihood of scleroderma development.
In most cases, systemic scleroderma debuts with Raynaud's syndrome, and therefore patients with this syndrome should be periodically examined by a rheumatologist for the timely detection of scleroderma. Further, arthritis and limited mobility of the fingers are attached, so differential diagnostics with rheumatoid arthritis is necessary. In a small proportion of patients, systemic scleroderma can manifest only muscle weakness. The main complications of scleroderma are circulatory disorders, deformities of the extremities, more than half of patients suffer from pulmonary fibrosis, which is manifested by shortness of breath, prolonged painful cough and pleural pain.
There are no precise diagnostic signs, and therefore, if there are conditions that tissue swelling is caused by scleroderma, then all possible pathologies must be excluded in order to make a diagnosis. The main diagnostic signs of scleroderma are scleroderma skin lesions, positive Raynaud's syndrome, joint-muscular syndrome, sclerotic and fibrotic changes of internal organs.
Additional diagnostic criteria - hyperpigmentation of the skin, telangiectasia, a sharp loss of body weight (by 10 and more kg), a significantly increased rate of erythrocyte sedimentation in a clinical examination of the blood. When biochemical blood test revealed hypergammaglobulinemia, the presence of antibodies to DNA and ANF (antinuclear factor) and a positive reaction to the rheumatoid factor. The presence of three reliable signs or three additional in combination with fibro-sclerotic changes in internal organs is the criterion for the diagnosis.
The earlier the scleroderma therapy is started, the less likely it is to spread. Uncomplicated forms of the disease usually pass by themselves. Systemic scleroderma requires long-term therapy, the main principles of which is the appointment of antifibrotic drugs - penicillamine, solesulfone. The drugs are taken on a prescribed schedule for 3-5 years, only in this case it is possible to prevent the damage to the internal organs.
Enzyme preparations and glucocorticosteroids are prescribed as indications for additional therapy. Local massage is shown, electrophoresis with dimethyl sulfoxide, laser therapy. All patients with systemic scleroderma are shown acupuncture, sulfurous and carbonic baths favorably influence the course of scleroderma.
The last method of treating scleroderma is stem cell therapy. With the introduction of stem cells into the human body, the protective properties of the body are significantly increased, the ability to form new vessels bypassing the damaged ones is activated. Stem cells also replace dead nerve cells, thereby stopping spasms of blood vessels and activating the process of tissue regeneration. Gradually there is a complete restoration of fibrotic changes in the internal organs, which prevents their sclerosis.