Hemophagocytic lymphohistiocytosis associated with dermatomyositis: a case report.

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is an uncommon life-threatening disorder of pathologic immune activation. HLH associated with autoimmune disease is recognized although the mechanism remains uncertain. HLH associated with dermatomyositis is very rare and just as rarely reported in medical literature.