Juvenile Dermatomyositis and Posterior Reversible Encephalopathy Syndrome: A Rare Association.

Juvenile dermatomyositis or JDM is a rare but debilitating pediatric disease that affects the proximal muscles and skin, as implied by its name. Although rare, it is the most common childhood idiopathic inflammatory myopathy (1). The estimated incidence is 3.2 cases per million children (2). The diagnosis is based on clinical signs and symptoms supported by investigations including, but not limited to, muscle biopsy, muscle enzymes and EMG studies. Bohan and Peter’s criterion is most widely used for diagnosis. It includes the presence of Gottron’s rash, raised muscle enzymes, proximal muscle weakness and suggestive findings on muscle biopsy (3). Treatment is done with a combination of steroid and immune-suppressing agents, mainly methotrexate (4). This report illustrates a case of JDM that presented with Posterior Reversible Encephalopathy Syndrome (PRES) and vision loss which are unusual presentations among children. Only a handful of studies have been published in existing literature where children with JDM presented with neurological symptoms