Anasarca as the presenting symptom of juvenile dermatomyositis: a case series

Abstract

Background: Juvenile Dermatomyositis (JDM) is an autoimmune disease that typically presents with classic skin rashes and proximal muscle weakness. Anasarca is a rare manifestation of this disease and is associated with a more severe and refractory course, requiring increased immunosuppression. Early recognition of this atypical presentation of JDM may lead to earlier treatment and better outcomes. Case presentation: We present two female patients, ages 11 years old and 4 years old, who presented to the ED with anasarca and were subsequently diagnosed with JDM. Both patients required ICU-level care and significant immunosuppression, including prolonged courses of IV methylprednisolone, IVIG, and Rituximab. Conclusions: Anasarca is a rare presentation of Juvenile Dermatomyositis, but it is important for clinicians to recognize this manifestation of the disease. Early recognition and treatment will lead to better outcomes in these children and hopefully decrease the need for prolonged hospitalization and ICU level care.