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Dermatomyositis is a systemic disease of skeletal, smooth muscle and skin. Etiology. The etiology is not known. Assume a viral (Koksaki B2) etiology of dermatomyositis. Provoking factors are insolation, cooling, trauma, pregnancy, drug intolerance, vaccination, stressful situations. Pathogenesis. A variety of immunopathological disorders. Clinic. The disease begins with an acute or subacute course, with a muscle syndrome in the form of myasthenia gravis, myalgia, arthralgia also develops, fever appears, with skin lesions, the clinical picture is in the form of dense, common edema. Skin lesions are diverse, often lilac paraorbital erythema with or without edema, erythema over extensor surfaces of the joint, sometimes with atrophic scars. The defeat of skeletal muscles is observed in the form of myalgia during movement and at rest, manifested in the form of soreness with pressure and increasing weakness. The muscles of the shoulder and pelvic girdles are tightened, enlarged, active movements are significantly impaired, the patient cannot sit on his own, lift his head from the pillow and keep it sitting or standing, raise limbs. With a large common process, patients are completely immobilized, and in severe cases they are in a state of complete prostration. In case of skeletal muscle damage, weakness, pain, swelling, muscle tightening, muscle hypotrophy, calcification appear. Visceral-muscular syndrome - damage to symmetrical, mainly proximal, muscle groups, facial muscles leads to facial maskiness, dysphagia develops in case of pharyngeal damage, and breathing and a decrease in vital capacity of the lungs, hypoventilation are disturbed in case of damage to intercostal muscles and diaphragm muscles. The oculomotor muscles can be affected with the development of diplopia, strabismus, bilateral ptosis of the eyelids, etc. Polyarthralgia during movement is manifested by a limitation of joint mobility up to the development of ankylosis, mostly due to muscle damage. Myocardial damage is observed of an inflammatory or dystrophic nature, which is manifested by persistent tachycardia and pulse lability, expansion of the borders of the heart to the left, with auscultation, muffling of heart sounds, systolic murmur at the apex, arterial hypotension. Damage to the lungs is associated with the underlying disease, more often it is caused by an infection to which patients are predisposed due to hypoventilation of the lungs.
The gastrointestinal tract is also involved in the process, anorexia, abdominal pains are noted, symptoms of gastroenterocolitis, hypotension of the upper third of the esophagus appear. Damage to the peripheral and central nervous system. Diagnostics. The criteria for the diagnosis of dermatomyositis are as follows. 1. Proximal muscle weakness for at least one month. 2. Myalgia for one month in the absence of sensitivity disorders. 3. The ratio of the concentration of creatine in the urine to the sum of the concentrations of creatine and creatinine in the urine in excess of 40%. 4. A significant increase in blood levels of creatine phosphokinase or transaminases in the absence of creatine phosphokinase or transaminases in the absence of other causes. 5. Degenerative changes in muscle fibers during biopsy. 4 signs - a reliable diagnosis, 3 signs - a probable diagnosis, 2 signs - a possible diagnosis. Diagnosis: on the basis of clinical, laboratory data, moderate leukocytosis with severe eosinophilia, a moderate increase in ESR, hypergammaglobulinemia are usually observed. The following studies are of great diagnostic value: a biochemical study of blood and urine, muscle biopsy, especially with subacute and chronic course (thickened muscle fibers are detected by transverse striation, fragmentation and dystrophy up to necrosis, a significant cellular reaction appears in the form of an accumulation of leukocytes, plasma cells, etc. . d.). Treatment. In acute and subacute course, glucocorticoids are shown in large daily doses (prednisone). After the effect is achieved, the dose of corticosteroids is reduced very slowly (half a tablet every 7-10 days), to a maintenance dose, against the background of delagil (0.25 g), plaquenil (0.2 g), 1 tablet after dinner. With the development of persistent remission, glucocorticoids can be completely canceled. In complex treatment, B vitamins, ascorbic acid are prescribed. With severe muscle fatigue, proserin and its analogues are prescribed in usual doses, ATP. With early treatment with adequate doses of corticosteroids in patients with acute dermatomyositis, a stable recovery occurs. In a subacute course, remission can be achieved with a maintenance dose of glucocorticoids. In chronic dermatomyositis, the disease acquires a wave-like course.
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This is a systemic progressive disease, characterized by a predominant lesion of striated and smooth muscles with impaired motor function, as well as skin. There are primary (idiopathic) and secondary (symptomatic) dermatomyositis, which develops with cancer of the internal organs. Primary dermatomyositis. Etiopathogenesis. The etiology of the disease is not completely
Dermatomyositis (DM) is a systemic inflammatory disease of skeletal and smooth muscles and skin; less involvement in the pathological process of internal organs is noted. In the absence of skin lesions, the term “polymyositis” (PM) is used. The essence of the disease consists in progressive severe necrotic myositis with a primary lesion of the muscles of the proximal extremities. As
Dermatomyositis is a systemic progressive disease, manifested mainly by damage to the striated and smooth muscles with impaired motor function, as well as skin integument in the form of erythema, edema, and a mild, but often visceral pathology. In 25-30% of patients, skin syndrome may be absent, in this case, to indicate the disease is used
Dermatomyositis (synonyms - Wagner’s disease, Wagner-Unferricht-Hepp syndrome) is a multifactorial disease characterized by widespread, mainly erythematous skin rashes and severe damage to the striated muscles. The disease is characterized by a chronically progressive course leading to disability due to muscle damage. Etiology and pathogenesis. IN
- a relatively rare disease with various, often erythematous, skin manifestations, severe systemic muscle damage and polymorphic visceral pathology, mainly myogenic, less often vascular origin. The main clinical manifestations In the clinical picture of dermatomyositis, skin and muscle lesions dominate. The most common and typical skin manifestation of dermatomyositis is
- DERMATOMYOSIS AND POLYMYOSIS
WG Bradtey Dermatomyositis and polymyositis are diseases of unknown etiology, characterized by skeletal muscle damage and accompanied by an inflammatory process without suppuration with a predominance of lymphocytic cell infiltration. The term "polymyositis" is used in cases where the skin is not involved in the pathological process, while the term "dermatomyositis" is used in
-general s-we: weight loss, fever -skin s-m: lilac paraorbital erythema, erythema over joints, periarticular edema, common; capillaritis - articular cm: arthritis, arthralgia - muscle cm: muscle weakness in the proximal limbs, myalgia, myositis, sclerosis and muscle calcification, muscle contractures - lung cm: vascular interstitial pneumonia, pleurisy
- Inflammatory myopathy
There are various inflammatory diseases in which skeletal muscle is involved: infectious myositis (see chapter 14), non-infectious inflammatory muscle diseases (polymyositis, dermatomyositis, etc., see chapter 5) and inflammatory diseases associated with diffuse systemic inflammatory
- RHEUMATIC DISEASES
Rheumatic diseases (systemic diseases of the connective tissue with immune disorders) are a group of diseases characterized by damage to the connective tissue in connection with a violation of immune homeostasis. • The group of rheumatic diseases includes rheumatism, rheumatoid arthritis, systemic lupus erythematosus, ankylosing spondylitis, systemic scleroderma, periarteritis nodosa,
Waardenburg Syndrome. See Waardenburg Syndrome. Wagner's disease See Dermatomyositis. Wagner-Unferricht-Hepp disease See Dermatomyositis. Valdenstrom macroglobinemia See Hyperviscosity syndrome. Wardenburg syndrome Wardenburg P. (Waardenburg R.) - Dutch ophthalmologist. It is transmitted by an autosomal dominant type. Includes lateral atopy of the medial angle of the palpebral fissure and the lacrimal opening,
- Classification of joint diseases.
1. Rheumatism (rheumatic fever). 2. Diffuse diseases of the connective tissue: 1) systemic lupus erythematosus; 2) systemic scleroderma; 3) diffuse fasciitis; 4) dermatomyositis; 5) Sjogren's disease; 6) rheumatic polyalgia; 7) recurrent polychondritis; 8) Titz disease. 3. Systemic vasculitis (angiitis, arteritis): 1) periarteritis nodosa; 2) granulomatous arteritis; 3) hyperergic
- LECTURE No. 9. Differential diagnosis of diffuse diseases of connective tissue in children. Clinic, diagnosis, treatment
Classification of rheumatic diseases. 1. Rheumatism. 2. Juvenile rheumatoid arthritis. 3. Ankylosing spondylitis. 4. Other spondyloarthropathies. 5. Systemic lupus erythematosus. 6. Vasculitis: 1) hemorrhagic vasculitis (Shacklein-Genoa); 2) periarteritis nodosa (polyarteritis in young children, Kawasaki disease, Wegener's disease); 3) Takayasu arteritis. 7. Dermatomyositis. 8. Scleroderma. 9.
- List of Accepted Abbreviations
AH - arterial hypertension HELL - blood pressure ADH - antidiuretic hormone CABG - coronary artery bypass grafting ALT - alanine aminotransferase ANF - antinuclear factor ATP - angiotensin converting enzyme ACT - aspartate aminotransferase BA - bronchial adrenergic hypertension - diabetes mellitus - diabetes mellitus - diabetes