Anasarca as the presenting feature of juvenile dermatomyositis

We report a 10-year-old girl with juvenile dermatomyositis who presented with generalized anasarca, an unusual initial manifestation, rarely reported. Anasarca has been linked with severity of the disease activity in some reports. The patient was treated with pulse methylprednisolone, methotrexate and intravenous immunoglobulin. Disease activity was completely controlled within 3 months of therapy. It is crucial for pediatricians to recognize generalized edema as a presenting feature of juvenile dermatomyositis in the absence of the other clinical and laboratory features of other common causes of anasarca. We advocate early diagnosis and prompt aggressive treatment to decrease morbidity.