Scleroderma systemic

Systemic scleroderma is a chronic disease of the connective tissue system and small vessels with widespread skin lesions (sclerosis, fibrosis), the connective tissue basis of internal organs. The reason is unknown. It is provoked by cooling, trauma, infection, vaccination, etc. The family genetic predisposition matters. Women get sick 3 times more often than men.

Symptoms and course

Usually the disease begins with Raynaud’s syndrome, joint pain, weight loss, fever, weakness, fatigue. The most characteristic symptom is skin lesions. At first, there is a dense widespread edema, later on – thickening and atrophy of the skin, especially pronounced on the face and extremities. Perhaps the appearance of ulcers and pustules on the fingertips, long-term non-healing, very painful. Nails are deformed, hair falls out up to baldness. Painful compaction, and then muscle atrophy, is accompanied by gross changes in the tendons: they shorten, which leads to the development of contractures that disrupt the activity of various joints. Pains appear in them, they are deformed. An x-ray examination reveals destruction – osteolysis of the terminal, and in severe cases, the middle phalanges of the hands, less often the legs. Lumps of calcium can be deposited in the thickness of the subcutaneous tissue. The disease also affects the cardiovascular system. There are pains in the region of the heart, shortness of breath, all kinds of violations of the heart rhythm and conduction. With immune inflammation of the vessels, gangrene, thrombophlebitis with trophic ulcers on the feet, legs, etc. can occur. Severe damage to internal organs is possible: lungs – pneumofibrosis, kidneys – “scleroderma kidney”, diffuse glomerulonephritis. Particularly characteristic is the violation of the passage of food through the esophagus, its expansion, detected by X-ray examination. The defeat of the nervous system is manifested by polyneuritis, vegetative instability (impaired sweating, thermoregulation), emotional (irritability, tearfulness and suspiciousness), insomnia. In rare cases, encephalitis and psychosis occur. Allocate sharp, subacute and chronic course of the disease. Changes in the blood are nonspecific. The level of hemoglobin decreases, the number of leukocytes, ESR may increase.


The diagnosis is confirmed by the detection of specific changes in the immunological status and by skin biopsy.


Large doses of glucocorticoid hormones (prednisolone), also D-penicillamine, cuprenil, delagil are used. For the treatment of Raynaud’s syndrome – nifedipine (Corinfar, Cordafen, Foridon). Symptomatic therapy is needed: lidase, B vitamins, vasodilators, physiotherapy (coniferous, radon, hydrogen sulfide baths, mud therapy, paraffin therapy, etc.), physiotherapy exercises, massage.

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