Polymyositis and dermatomyositis are uncommon Rheumatic diseases (polymyositis) or muscles and skin (dermatomyositis) are characterized by inflammatory and degenerative changes in the muscles. The specific skin features are Gottron papules over the knuckles and a periorbital heliotropischer rash. Some common manifestations include symmetrical muscle weakness, tenderness, and later atrophy, proximal limb musculature preferred concerning. Complications include organ involvement and associated cancers. The diagnosis is made by typical clinical findings and pathological muscle studies (incl. Muscle enzymes, MRI, EMG and biopsy). The treatment consists of corticosteroids, usually in combination with immunosuppressants or i.v. Immunoglobulin.

The ratio of women to men is 2: 1. An occurrence is at any age possible, most commonly between the ages of 40 and 60 years of age, in children between the 5th and 15th

Polymyositis and dermatomyositis are uncommon Rheumatic diseases (polymyositis) or muscles and skin (dermatomyositis) are characterized by inflammatory and degenerative changes in the muscles. The specific skin features are Gottron papules over the knuckles and a periorbital heliotropischer rash. Some common manifestations include symmetrical muscle weakness, tenderness, and later atrophy, proximal limb musculature preferred concerning. Complications include organ involvement and associated cancers. The diagnosis is made by typical clinical findings and pathological muscle studies (incl. Muscle enzymes, MRI, EMG and biopsy). The treatment consists of corticosteroids, usually in combination with immunosuppressants or i.v. Immunoglobulin. The ratio of women to men is 2: 1. An occurrence is at any age possible, most commonly between the ages of 40 and 60 years of age, in children between the 5th and 15th etiology The cause an autoimmune reaction of muscle tissue in genetically susceptible people seem to be. A family history is known as a genetic association with the HLA subtypes DR3, DR52 and DR6. The trigger may act viral myositis and accompanying cancer. Picornavirusähnliche structures were detected in muscle cells, but their significance is unknown. Viral pathogens can cause similar diseases in animals. The known association of dermatomyositis (less polymyositis) with cancers suggests that tumors can induce myositis as a result of an autoimmune response to a common antigen in muscle and tumor. Pathophysiology Typical pathological changes in both diseases include cellular damage and atrophy of different inflammation degree. The muscles of the hands, feet and face is less affected than the rest of skeletal muscle. For infestation of muscles in the pharynx, upper esophagus and sometimes the heart can come to the impairment of organ function. Inflammatory processes may also occur in joints and lungs, especially in patients with Antisynthetaseantikörpern. Dermatomyositis is characterized by immune complex deposits in the vasculature and is known as complement-vasculopathy. In contrast, the most important pathophysiological abnormality in polymyositis is a direct T cell-mediated muscle injury. The classification myositis is divided into several subtypes: The primary idiopathic polymyositis can occur at any age and not affects the skin. The primary idiopathic dermatomyositis is similar to the primary idiopathic polymyositis, but also affects the skin. Polymyositis or dermatomyositis in association with tumors can occur at any age, but is most common in older patients; the tumor can manifest before and after the myositis up to 2 years. Dermatomyositis in childhood may be associated with a systemic vasculitis. Polymyositis or dermatomyositis can occur with an associated disease such as progressive systemic sclerosis, a MCTD, a RA, SLE or sarcoidosis. The inclusion body is a distinct disease which has some clinical similarities with chronic idiopathic polymyositis. However, it occurs usually in the elderly, often affects the distal muscles (z. B. hands and feet), lasted longer, is less responsive to therapy and has histological differences. Symptoms and signs The disease can begin acutely (v. A. In children) or gradual (rather in adults). Polyarthralgia, Raynaud’s syndrome, dysphagia, pulmonary symptoms and general symptoms (v. A. Fever, fatigue and weight loss) can also occur. Muscle weakness may progress over weeks to months. However, it requires the destruction of 50% of the muscle fibers to cause symptomatic muscle weakness, d. h., this manifestation signals an already advanced myositis. Patients describe difficulty raising arms over his shoulders, climbing stairs or getting up from a sitting position. Patients can even be wheelchair-bound or bedridden due to the weakness of the pelvic and shoulder girdle muscles. The neck flexors may be so badly affected that the head can not be lifted from the pillow. The infection of the pharynx and upper esophagus muscles can make it difficult to swallow and predispose to aspiration. The muscles of the hands and feet and face remain largely spared. Contractures in the extremities may occur. In approximately 30% of patients experience joint involvement including polyarthralgia or polyarthritis, with swelling, effusion and other signs of non-destructive arthritis. Most, however, the joint involvement falls out mildly. It is found most often in a direction indicated by anti-Jo-1 or other Antisynthetaseantikörper special form. Apart from the participation of the pharynx and upper esophagus organ involvement in polymyositis is much rarer to find than in other rheumatic diseases system such. B. SLE or systemic sclerosis. interstitial pneumonitis can stand in the foreground especially in patients with Antisynthetaseantikörpern. Arrhythmia, especially cardiac conduction disorders or ventricular dysfunction may occur. In children with Begleitvaskulitis gastrointestinal symptoms such as hematemesis, melena and ischemic bowel perforation can be added. The lesions occur in dermatomyositis have a dark erythematous character. A purple periorbital edema (heliotropic rash) is relatively specific for the disease. Elsewhere, the rash may slightly raised and scaly smooth or occurring; he shows up at the front, a V-shape in the neck-shoulder portion (on the thorax or back, on lower arms and legs, at the elbow and knee, the medial malleoli and to the radiodorsalen sides of the PIP, and MCP joints Gottron -Papeln) – ie a relatively specific finding. Base and sides of the fingernails may be hyperemic and thickened. dermatitis with exfoliation and cracks can occur on the radial side of the finger. The primary lesions disappear completely, but can be followed by secondary phenomena (z. B. brownish pigmentation, atrophy, scarring, vitiligo). A rash on the scalp psoriasis appears shaped and highly itchy. Subcutaneous calcifications are v. a. to find in affected children. Heliotropic rash © Springer Science + Business Media var model = {thumbnailUrl: ‘/-/media/manual/professional/images/507-heliotrope-rash-s103-springer-high_de.jpg?la=de&thn=0&mw=350’ imageUrl ‘/-/media/manual/professional/images/507-heliotrope-rash-s103-springer-high_de.jpg?la=de&thn=0’, title: ‘heliotropic rash’, description: ‘ u003Ca id = ” v37892765 “class = ” anchor “” u003e u003c / a u003e u003cdiv class = “” para “” u003e u003cp u003eDiese illustration shows a purple rash around the eyes and in the nasolabial folds and chin a patient with dermatomyositis u003c / p u003e u003c / div u003e ‘credits’. © Springer Science + Business Media’

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