A case of multicentric reticulohistiocytosis is presented, with typical clinical, roentgenologic, and histologic features. The characteristic giant cells were seen in skin nodules, mucosal nodules, synovial and bony lesions of the joints, and tenosynovial cysts. Corticosteroid therapy produced a subjective improvement, but the arthritis mutilans did not improve.
Laboratory studies showed iron‐deficiency anemia, hypertriglyceridemia (hyperprebetalipo‐proteinemia), a low response to the unstimulated and stimulated nitroblue tetrazolium test of neutrophils, and a decreased activity in the glucose‐6‐phosphate dehydrogenase of polimorphonuclear leucocytes before corticosteroid therapy. The histochemical examination of the material in the giant cells showed a lipid (a phospholipid and a neutral fat), and a polysaccharide attached to a protein (a muco‐ or glycoprotein). Electron microscopic studies of the giant cells indicated that their cytoplasm contained numerous dense granules clustered in the vicinity of the Golgi apparatus. In some areas the granules appeared to be developing from the Golgi vesicles and cisternas.
It seemed likely that the storage material was produced within the giant cells. We also discussed the pathogenesis of this disorder.