Severe macrophage activation syndrome and central nervous system involvement in juvenile dermatomyositis.

摘要

Juvenile dermatomyositis (JDM) is a rare autoinflamma-tory vasculopathy that presents with proximal muscle weakness and dermatitis. Periorbital oedema may be associated with this disease but generalized oedema is uncommon (1). Central nervous system (CNS) involvement and macrophage activation syndrome (MAS) are rarely reported in JDM (1, 2). We present a case with both complications and an unusual presentation with severe generalized oedema.