Poikilodermatomyositis (poikilodermatomyositis; from the Greek. poikilos - and-white, spotty; derma, dermatos - skin; mys, myos - muscle; synonym syndrome Petra - Klia) is a chronic disease of the muscles and skin, flowing with minor remissions.
Muscular lesions begin with edema and significant pain, later develop weakness and atrophy. Since the muscles of the shoulder and pelvic region, the process of gradually expanding to extensive muscle groups, while atrophy can develop sclerotic changes. On the skin occur gradually increasing in number and size spotted atrophy, and telangiectasia and pigmentation, which may precede the development swollen pink foci. Skin thinning, slightly scaly. These changes can capture large areas of the skin of face, neck and chest. In some patients poikilodermatomyositis observed the deposition of calcium salts in the muscles. Histologically - atrophy of the epidermis, the degeneration of collagen fibers, muscle edema, hemorrhage, round-cell perivascular infiltration, hyaline degeneration of collagen, sclerosis and muscular atrophy beams.
The General condition of patients with a significant extension of the process can be very heavy, accompanied by changes of the heart and lungs. Poikilodermatomyositis may develop in patients weakened by severe infectious diseases (tuberculosis, strep sepsis). Differential diagnosis of a lupus (see), dermatomyositis (see) and chronic Trofimuk the atrophicans (see) can be very complex. Some authors consider poikilodermatomyositis and dermatomyositis different manifestations of the same disease. Note the transfer from one form of defeat to another.
Treatment with corticosteroids, antibiotics (streptomycin, penicillin, terramycin), p-aminobenzoic acid (1.0-1.5 g / day), vitamin E, advanced antimalarial drugs.