Juvenile Dermatomyositis with Generalized Edema

A 7-year-old girl presenting with an 8-week history of fatigue, myalgia, dyspnea, and generalized, nonpitting edema of the extremities, face, chest, and abdomen, is reported from the Walter Reed Army Medical Center, Washington, DC. A heliotrope and malar rash had developed, and muscles were tender to palpation. A severe proximal muscle weakness, elevated serum muscle enzymes, and heliotrope rash were characteristic of juvenile dermatomyositis (JDM). Other diseases characteristic of generalized anasarca, such as nephrosis, liver disease, hypothyroidism, and malignancy, were excluded. Seventeen additional cases of JDM and anasarca were cited in the literature. Response to oral steroids was poor, and high dose long duration IV therapy was needed. [1]