Juvenile dermatomyositis: A review of clinical features and management

Juvenile dermatomyositis is the most common of the idiopathic inflammatory myositis of childhood. It is characterised by a pathognomic skin rash along with proximal muscle weakness. It may also affect organs other than the muscle and skin. Calcinosis and lipodystrophy are complications of long-standing untreated or poorly treated disease, associated with chronic morbidity. A variety of environmental and genetic risk factors are implicated in its pathogenesis. Up-regulation of genes by type-I interferon appears central. Corticosteroids along with methotrexate are the mainstay of therapy. Disease outcome is in general good.