Abstract
BackgroundJuvenile 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 presentationWe 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.ConclusionsAnasarca 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.
Highlights
Juvenile Dermatomyositis (JDM) is an autoimmune disease that typically presents with classic skin rashes and proximal muscle weakness
We describe two patients with a final diagnosis of Juvenile Dermatomyositis who presented with anasarca, both of whom required intensive care unit (ICU)-level care
Once the pathology results returned consistent with JDM, treatment was initiated with pulse dose (30 mg/kg/day) of IV methylprednisolone (IVMP)
Summary
Anasarca is a rare presentation of Juvenile Dermatomyositis, but it is important for clinicians to recognize this manifestation of the disease.
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