POIKILODERMATOMYOSITIS (poikilodermatomyositis; grech, poikilos motley + dermatomyositis; synonym generalized miositis of Petzha — Klezha) — the disease which is characterized by damage of skin with existence of teleangiectasias, a nevus pigmentosus and sites of an atrophy, and also systemic lesion of skeletal muscles. The item — option dermatomyositis (see), treats group collagenic diseases (see). Petzhem and Klezha (G. Petges, G. Clejat) in 1906 is described for the first time. Occurs at any age, a thicket at women.
Etiology and pathogeny are found out insufficiently.
Gistol, changes of skin correspond to a picture poikilodermas (see). In hypodermic cellulose the centers can be found calcification (see). Damage of muscles similarly observed at a dermatomyositis with dominance of processes of dystrophy of myocytes, infiltrations, fibrosis and a sclerosis of intersticial fabric; the thickening of an endothelium and a basal membrane of intramuscular arterioles and capillaries is noted.
Clinical picture it is characterized by generalized damage of skin and muscles. Diversity of skin changes is typical. Against the background of a hyperpegmentation the centers of a depigmentation, multiple are noted teleangiectasias (see), especially on a face and a breast, thinning of skin, sometimes a peeling, an itch, sites hyperkeratosis (see). Trophic changes of nails, a hair loss, an atrophy of mucous membranes are frequent.
Damage of muscles causes weight of a condition of patients. Systemic lesion of cross-striped muscles, preferential proximal departments of extremities with the increasing weakness and restriction of movements is characteristic. Also widespread adjournment in muscles of salts of calcium can be a cause of infringement of movements.
The visceral pathology inherent to a dermatomyositis (damage of a myocardium and other bodies), is expressed unsharply. Laboratory indicators of activity of an inflammation are more often normal. Only at certain patients the eosinophilia, moderate increase in levels of a kre-atinfosfokinaza and transaminases in blood serum is observed. The disease has usually hron, a current.
Diagnosis The item is based on characteristic changes of skin in combination with signs of a polymiositis (see. Miositis ), is confirmed by data of a biopsy of skin and muscles. Differential diagnosis is carried out with system scleroderma (see) and system lupus erythematosus (see), and at dominance of changes from skin — with Jacobi's poikiloderma (See. Poikiloderma ).
Treatment similarly carried out at a dermatomyositis: glucocorticosteroids, non-steroidal anti-inflammatory drugs (acetilsalicylic to - that, indometacin, etc.), disodium salt etilendiaminotetrauksusny to - you intravenously and method of an electrophoresis in the presence of kaltsinat, fortifying therapy, massage, LFK.
Forecast is defined by weight of damage of muscles. At timely begun treatment the forecast rather favorable.
Bibliography: Mashkilleyson L. N. Private dermatology, page 374, M., 1965; Tareev E. M. Collagenoses, page 267, M., 1965; Petges e. Poikilodermia et polymyosite (poikilodermato-myosite), Bull. Soc. franQ. Derm. Syph., p. 817, 1929.
H. G. Guseva.