Multicentric reticulohistiocytosis: systemic macrophage disorder.

Multicentric reticulohistiocytosis is a rare multisystem disorder that reflects a reactive inflammatory response to an undetermined stimulus. While the disease is characterized as a dermatoarthritis, multiple organ systems including cardiac and skeletal muscle, the pleura and gastrointestinal tract have been involved in reported cases. The synovitis can be quite destructive with arthritis mutilans developing in a substantial percentage. The dermatitis may be particularly disfiguring when the face is involved. This chapter describes the clinical and laboratory features of the 33 cases of MRH previously reviewed by Barrow and Holubar and an additional 33 cases that have appeared in the medical literature since that report. We note an apparent decline in frequency of some manifestations of MRH. This may be due in part to the nature of the recent reports which often present a brief clinical report and focus primarily on specific disease associations, unusual manifestations, new organ system involvement or treatment regimens. The primary cell involved in the reactive inflammatory response of MRH is the phagocytic tissue histiocyte (macrophage). While uncontrolled proliferation of these reticulohistiocytes is seen in several infectious and malignant conditions there is presently no direct evidence of a particular organism or neoplasm involved in the aetiopathogenesis of MRH. There is evidence of tuberculosis exposure in one third of cases with active tuberculosis present in 5%. Likewise, malignancies are reported concomitantly with MRH in 15-28% of cases. The therapeutic trend in MRH is to treat early and aggressively to prevent the devastating arthropathy and disfiguring cutaneous sequelae. This recommendation, however, is largely based on anecdotal reports and thus the physician encountering a case of MRH needs to proceed with circumspection.