A Rare Case of Evans Syndrome Associated with Underlying Dermatomyositis Without Generalized Subcutaneous Edema: A Case Report

Abstract

Background: Primary Evans Syndrome is a typically idiopathic systemic illness associated with a number of autoimmune conditions and lymphomas and characterized by Coombs-positive warm-agglutinin Autoimmune Hemolytic Anemia (AIHA) and immune thrombocytopenia. The disease can be severe and prolonged with multiple recurring disease flares. Its association with dermatomyositis remains exceedingly rare and all reported cases have been accompanied by generalized subcutaneous edema. Case Report: We report a case of a 24-year-old male with a decade-long medical history of Evans Syndrome who presented to the emergency room with a one-week history of shortness of breath and fatigue. An extensive workup revealed severe anemia, thrombocytopenia and a left hemothorax. Active intervention included video-assisted thoracoscopy with decortication, blood products and autoimmune investigation. He was ultimately diagnosed with dermatomyositis after an electromyograph showed findings consistent with demyelinating peripheral polyneuropathy of motor nerves. Conclusion: Evans syndrome associated with dermatomyositis is extremely rare and this is to our knowledge the first reported case of Evans syndrome associated with dermatomyositis without generalized subcutaneous edema.