Scleroderma

Description:

The scleroderma — a chronic inflammatory disease of connecting fabric which in classical cases affects skin, but can take also digestive tract, heart, lungs, kidneys and synovial fabrics. The skin defeats which are a cardinal symptom of a disease can be focal, linear or generalized, with symmetric distribution of skin elements. The last form usually is followed by systemic lesions (the progressing system sclerosis) and is typical for adult patients. At children the scleroderma usually has the focal form; systemic lesions are observed seldom.
Scleroderma - the disease of connecting fabric which is characterized by its consolidation (sklerozirovaniye). Specialists distinguish two forms of a disease: limited (focal) at which it is possible to hope for a favorable outcome, and a system (widespread) form of a scleroderma which hardly will respond to treatment. Women are ill a scleroderma several times more often than men.

Scleroderma symptoms:

Allocate a focal and linear scleroderma. The first precursory symptoms of a disease are focal damages of skin and hypodermic fabrics. These elements often are located in a linear order, similar to the course of peripheral nerves, and can preferential be localized on one side of a body. At early stages sites of damage of skin have slightly erythematic appearance, are edematous or look atrofichny and brilliant.
The patient can complain of pain or feeling of a pricking. In process of progressing of a disease sites of defeat are exposed to an induration, their edges get a lilac shade and sometimes rise, and the central part takes a pale waxy form. Skin elements increase in the peripheral direction and can merge among themselves, taking all extremity or extensive sites of a trunk. In sites of defeat the expressed cicatricial changes and fibrosis develop, skin fabrics are densely accustomed to drinking with the subject structures. This process is sometimes so expressed that leads to restriction of growth of the affected extremity and emergence of the spoiling contractures. Zones of chronic defeat can be giperpigmentirovana and are depigmented.
The extensive focal scleroderma of the left leg which caused scarring, its shortening and flexion contractures.
Skin is brilliant, spots of a hyperpegmentation and vitiligo on affected areas are visible.
Active pathological process can stop in several months or years or to last inertly for many years. In the absence of systemic lesions the forecast for life favorable. The progressing system sclerosis. Skin defeats have symmetric character. They are localized on hands, feet and distal departments of extremities, sometimes on a trunk and a face. As well as at focal forms of a disease, the induration, pigmental changes and soldering of the struck skin fabrics can take place. In some cases Reynaud's phenomenon and skin ulcers are observed. The synovitis, especially brush joints, can remind a pseudorheumatism picture; are sometimes observed tenoinovit also small knots on the course of tendinous vaginas.
Pathological process can take digestive tract, heart, lungs and kidneys. System displays of a disease, especially damages of kidneys, heart and lungs, sometimes lead to a lethal outcome. Dysfunction of a gullet can be the cause of chronic aspiration pneumonia. In some cases there is heavy hypertensia.
Reynaud's syndrome - the most frequent and precursory symptom of a system scleroderma. It is characterized by sudden emergence of paresthesia (feeling of numbness, crawling of goosebumps) in the area II-IV of fingers of brushes, feet, their sharp blanching, fingers during an attack cold. Upon termination of an attack there are pains, feeling of heat in fingers, skin is hyperemic. At a system scleroderma Reynaud's syndrome takes not only fingers of hands, feet, but also lips a tip of language, a part of the face. Vasospastic reactions it is mighty it is observed in lungs, heart, kidneys.
Datas of laboratory. Specific laboratory tests of a scleroderma do not exist. SOE is often not changed. Both at focal, and at the disease disseminated forms rhematoid factors and antinuclear antibodies are found. By means of X-ray inspections disturbances of motility of a gullet and a small bowel can come to light. Functional researches of lungs, an electrocardiography and a X-ray analysis of a thorax reveal defeats of cordial and respiratory systems. At damage of kidneys changes in urine and renal failures are noted.
Diagnosis. At focal forms of a scleroderma and the progressing system sclerosis the clinical picture is very characteristic. Superficially the disease can resemble to some extent a dermatomyositis, however lack of a miositis and the rash characteristic of a dermatomyositis allows to otdifferentsirovat these states. At a focal form of a scleroderma there can be a suspicion on a necrosis of a hypodermic fatty tissue and not purulent panniculitis of Weber — Krischena, however on a current and histologic changes these states differ from a scleroderma.

Scleroderma reasons:

In development of a scleroderma the major role is played by the inborn inferiority of immune system leading to autoimmune reactions. The contributing factors are catarrhal diseases, bacterial and viral infections, chronic diseases, including allergic, diseases of nervous and endocrine systems, the work connected with jolting, vibration, influence of harmful substances, stay on cold.
Under the influence of all these reasons regulation of work of small vessels is broken, their permeability increases, in surrounding fabrics hypostasis develops, collagen is produced that promotes growth of the connecting fabric replacing fabric of bodies and dysfunction of these bodies.

Treatment of the Scleroderma:

Specific treatment does not exist. Attempts to treat a scleroderma the most different means, including corticosteroids, salicylates, chelate agents, chloroquine, radiation, a dimethyl sulfoxide, paraaminobenzoic acid, Penicillaminum and cytostatics were made. A little noticeable effect did not manage to achieve. Surgical excision of affected areas of skin at a focal scleroderma does not lead to a stop of pathological process. At crushing systemic lesions it is possible to try to apply corticosteroids, Penicillaminum or cytostatics. Locally applied corticosteroids to treatment of focal defeats. For the prevention or minimizing of obezdvizhushchy contractures already at early stages of a focal scleroderma the intensive physiotherapy exercises are shown.