The authors describe a patient with acute onset dermatomyositis, „probable“, according to criteria of Bohan and Peter, as well as the development of the disease in the course of 21 months. A special feature was the decline of visual acuity (practically to the level of blindness). The cause was severe retinopathy with dominance of macular oedema, „cotton wooll“ spots and haemorrhages,as apparent from photographs of the fundus and findings obtained by fluorescein angiography. The problem of initial treatment was resistance to megadoses of glucocorticoid (i.v. pulses). A favourable change occurred only after i.v. administration of polyvalent immunoglobulin ina total dose of 600 mg/kg body weight. Then the authors observed the unique phenomenon of regression of retinal oedema incl. the macula, of the haemorrhages and „cotton wooll“ spots. Consistent with it vision off the right eye improved to 1.0 and the left eye to 0.66 in the course of 11 months. Stabilized retinal neovascularizations persist. On account of the latter the authors treated the retina by laser panretinocoagulation.

        Key words: dermatomyositis, macular oedema, „cotton wooll“ spots, retinal hae- morrhages