Dermatomyositis

Dermatomyositis is a systemic disease of skeletal and smooth muscles and skin. The presumed root cause of the disease is viral (Coxsackie B2 virus). Provoke the disease cooling, prolonged exposure to the sun, pregnancy, drug intolerance. In 20-30% of cases, dermatomyositis is observed in patients with various tumors. The mechanism of the development of the disease is disturbances in the immune status. Women get sick 2 times more often than men, with two age peaks (transitional age, menopause). There may be a family genetic predisposition.

Symptoms and course

The disease begins more or less acutely with damage to the muscular system (muscle weakness, muscle pain), joint pain, fever, skin lesions (various rash, itching, patchy redness, increased keratinization, increased or sharply reduced pigmentation, calcium deposition in thicker skin or subcutaneous fat), dense widespread edema. In the future, the disease acquires a relapsing course. Damage to the skeletal muscles progresses – they are painful during movement and at rest, the pain increases with pressure on them, muscle weakness increases. The muscles of the shoulder and pelvic girdle thicken, increase in volume, their active movements are disturbed, patients cannot sit down on their own, raise their heads from the pillow. The defeat of facial muscles leads to a mask-like face, pharyngeal and respiratory – to violations of swallowing and breathing, which is also complicated by frequent pneumonia. If the muscles of the eyes are affected, then double vision, strabismus, bilateral ptosis (drooping of the upper eyelid) develops. Involvement of the heart muscle in the process (myocarditis or myocardial dystrophy) is manifested by a persistent increase in heart rate, a decrease in pressure, the appearance of systolic murmur, and can lead to the development of heart failure. A third of the patients have Raynaud’s syndrome (whitening and blue of the fingers during cooling). Appetite is reduced or absent, there are abdominal pains, gastrointestinal bleeding, intestinal obstruction may develop. There are 3 forms of the course of dermatomyositis: acute with rapid development of symptoms (may result in death after 36 months due to severe damage to the lungs and cardiovascular system), subacute and chronic.

Recognition is based on a biochemical test of blood and urine (the level of the enzyme creatine phosphokinase), but the main role belongs to the detection of specific changes in the biopsied muscle.

Treatment

In the presence of a malignant tumor, its removal leads to a lasting improvement. It is necessary to use large doses of glucocorticoid hormones for a long time (prednisolone, methylprednisolone). It is possible to take delagil, plaquenil, indomethacin, brufen, butadione; vitamins of group B, ascorbic acid are important. With severe muscle weakness, prozerin, anabolic steroids (nerabol, retabolil) are used. After the inflammation subsides – physiotherapy exercises, massage.