Scleroderma (synonym for dermatosclerosis) is a progressive disease characterized by densification of the skin, and with a systemic nature of the process and damage to the internal organs. In the prevalence of the process, scleroderma is divided into systemic, when many organs and tissues are affected, and limited, in which there are mainly focal skin changes. At the heart of the disease are changes in connective tissue , in particular collagen fibers, with the development of sclerosis and obliterative lesions of arterioles.
Systemic scleroderma (synonym: universal, generalized, diffuse, progressive scleroderma, systemic progressive sclerosis) is a serious disease with a polymorphic clinical picture involving the defeat of the skin, joints, internal organs (heart, lungs, digestive tract, kidneys), against vascular and trophic violations.
Systemic scleroderma refers to the group of collagen diseases (see) or diffuse connective tissue diseases (according to WHO nomenclature). In the etiology, genetic and infectious factors may be important. The provocative role of cooling, trauma, foci of chronic infection, intolerance of drugs, endocrine disruptions (abortion, childbirth , menopause ) is known. The pathogenesis of the disease is complex and insufficiently studied. Great importance is attached to the pathology of collagen (increased biosynthesis and fibrillation) and immune (autoimmune) disorders, unfolding against the background of altered regulatory functions of the nervous and endocrine systems.The
Fig. 1. Diffuse scleroderma (masklike face). Fig. 2. Sclerodactyly (clerodermic lesion of the skin of the hand).
Clinical symptoms . Women predominate, mostly in the age of 20-50 years. The disease usually develops gradually: there are vasomotor disorders according to the type of Raynaud's syndrome (numbness and whitening or blueing of the fingers), arthralgia, tight swelling of the skin, less often - damage to the internal organs.
The defeat of the skin, which goes through the stages of edema, densification and atrophy, is noted in most patients and is localized mainly in the face (masculinity) and brushes - sclerodactyly (Figures 1 and 2). Often observed hyperpigmentation of the skin and telangiectasia on the face and chest. The phenomena of Raynaud's syndrome can be accompanied by pronounced trophic changes, repeated ulceration at the fingertips; sometimes develops gangrene of fingers. Articular syndrome - from mild arthralgia to severe joint deformity with the development of persistent contractures - is observed in most patients. In a part of patients with systemic scleroderma, deposition of calcium salts (calcinosis) in the subcutaneous tissue of the fingers and periarticular tissues is noted - the Tibiorge-Weissenbach syndrome. Many patients have osteoporosis, but osteolysis of the nail, and sometimes middle phalanges of the fingers, less often of the legs, with a shortening and deformation of the corresponding fingers is especially characteristic.
Lung infection is noted in most patients and is characterized by the gradual development of basal (compact, less often cystic) pneumosclerosis . There is a lesion of the myocardium in the form of focal or diffuse cardiosclerosis ; rarely affects the endocardium with the formation of scleroderma, more often mitral, heart disease, there are changes in the pericardium.The
The defeat of the gastrointestinal tract (often the esophagus ) is observed in about half of the cases and is characterized by impaired swallowing, heartburn, abdominal pain, persistent constipation, and sometimes diarrhea . The kidneys are relatively infrequent, but with acute and subacute course of the disease, it is possible to develop acute renal failure .
Often disrupted the endocrine (thyroid, sex glands, adrenal glands) and nervous (often peripheral and vegetative) systems. There is a loss of hair and a significant weight loss (up to 10-20 kg or more).
Quite often observed hyperproteinemia; in acute and subacute course of the disease - accelerated ROE , positive diphenylamine test and other signs of activity of the process.
Depending on the speed of progression and activity of the process, one distinguishes between acute (rarely observed), subacute and chronic course of the disease.
The prognosis for acute and subacute forms of scleroderma is more often unfavorable, in chronic cases it is much better.
Treatment . In acute and subacute flow, corticosteroids are used, preferably prednisolone , for a long time (months, often years). Assign the drug to 20-30 mg per day, followed by a lower dose. At the same time, derivatives of aminoquinoline - hingamin (chloroquine, delagil, resochin) 0.25 g after dinner, sometimes salicylates (more often acetylsalicylic acid 2-3 g per day), repeated courses of vitamins B1 and B6 intramuscularly (in spring and autumn) are used simultaneously. In case of violations of peripheral circulation and trophic - repeated courses of angiotrophin 1 ml for 10-15 days or depopadutin 1 ml for 30 days intramuscularly; disodium adenosine triphosphate in 1 ml of 1% solution intramuscularly for a month; with the deposition of secondary infection - antibiotics.
In chronic forms of scleroderma, these agents are also used, with the exception of corticosteroids; In addition, treatment with repeated courses of lidase at 64 units in 1 ml of 0.5% solution of novocaine subcutaneously every other day, 12-14 injections per course is indicated. Assign electrophoresis with hyaluronidase, paraffin baths, balneotherapy (hydrogen sulphide, radon baths), massage and physiotherapy .
Prevention of systemic scleroderma consists in the reasonable hardening of the body, the exclusion of provoking factors, the employment of patients and systematic observation for the purpose of supporting therapy, preventing relapses and further generalization of the process.