AN UNUSUAL PRESENTATION OF JUVENILE DERMATOMYOSITIS

Juvenile dermatomyositis(JDM) is a rare autoimmune disease characterised by inflammation of the muscle, connective tissue , skin, and small nerves. Although localized oedema is a common feature of JDM , generalised oedema has been reported rarely. In this article, we report a 4yr old boy with JDM presenting with generalised oedema. Of the diagnostic criteria of JDM , severe symmetric weakness of proximal musculature, characteristic cutaneous changes, elevated muscle enzymes and ruling out the other causes of anasarca, we came to a diagnosis of JDM . Our patient improved with methylprednisolone and methotrexate. We conclude that JDM should be suspected in children presenting with anasarca in the absence of laboratory parameters of other causes of generalised oedema with heliotrope rash with muscle weakness