Idiopathic lipogranulomatosis of spleen and periaortic lymph nodes with noncaseating granulomas of liver.

An unusual case of acute febrile illness clinically thought to be a manifestation of either granulomatous hepatitis or Hodgkin's disease is described. The spleen and enlarged periaortic lymph nodes at the level of the spleen revealed multiple noncaseating granulomas containing vacuoles and associated multinucleated giant cells. The lesions appeared to be consistent with lipogranulomas. The liver contained multiple noncaseating granulomas. The clinical history and analyses of tissue samples failed to document the presence of exogenous lipid. There was no evidence in support of a viral, fungal, or bacterial cause for any of the known granuloma-forming diseases. The patient recovered on steroid therapy and has remained well for one year following the onset of the acute illness. The possibility that this case represents lipogranulomatous pseudosarcoid of the spleen and periaortic lymph nodes or a variant of sarcoid cannot be excluded.