Abstract
Multicentric reticulohistiocytosis (MRH) is a rare systemic disease of unknown etiology: it is characterized by two major symptoms―proliferative, destructive polyarthritis and nodular lesions with the proliferation of histiocytes of the skin and mucous membrane. We had an opportunity to conduct an autopsy on a 54-year-old woman with MRH who suddenly succumbed to multifocal myocardial infarction. The patient had developed polyarthralgia, muscle weaknessof the extremities and skin symptoms. Initially, the possibility of dermatomyositis was considered but she was non-responsive to prednisolone, while the cutaneous nodules at her elbow were enlarged and destructive arthritis progressed. In spite of implantation of a permanent pacemaker, she eventually succumbed to cardiac arrest. At autopsy, diffuse infiltration by swollen histiocytes was noted in the synovium, trachea, lungs, spleen, pancreas, esophagus, small intestine and urinary bladder. However, the cardiac muscle was totally free of cell infiltration. The major cause of death was hemorrhagic infarction of the ventricular septum. The literature yields only one description of MRH that was observed at autopsy. We believe that this experience is extremely rare.
