Cardiac and systemic complications in multicentric reticulohistiocytosis

A 75-year-old man with a short history of cutaneous lesions of multicentric reticulohistiocytosis, preceded by a few months of a symmetrical polyarthritis is described. Within 5 months of onset of symptoms, he developed congestive cardiac failure secondary to pericardial involvement by the disease and succumbed despite therapy with cyclophosphamide and methylprednisolone. Post-mortem revealed the true extent of the disease, with nodules seen in the epiglottis and aryepiglottic folds, duodenal mesentery, pleura, pericardium and myocardium. Although the hallmarks of the disease are the papulonodular skin lesions, together with a severe, sometimes mutilating polyarthropathy, its widespread systemic nature is not often appreciated. We review five other cases in the literature with pericardial involvement and discuss aids to earlier diagnosis by synovial fluid cytology; gallium scanning is discussed as a potentially useful means of detecting the extent of systemic involvement in multicentric reticulohistiocytosis.