[Multifocal fibrosclerosis. 3 cases with skeletal and nervous system involvement].
Three cases of multifocal fibrosclerosis are reported. In the first case the clinical symptoms were diabetes insipidus associated with nodular pulmonary infiltrates and a retroperitoneal mass. In the second case, respiratory failure together with nodular pulmonary infiltrates dominated the picture. The third case was characterized principally by neurological symptoms and paraparesia secondary to the compression of the spinal cord by an intra-rachidial growth. In cases 1 and 3, X-ray studies of the skeleton showed a very peculiar involvement of bone shafts. Anatomical studies either by repeated biopsies or at autopsy showed that in all three cases the disease was characterized by disseminated fibrosclerosis. In the first two cases the involvement was most marked in the retroperitoneum, mediastinum, lungs and bones; in the third case the involvement was demonstrated in the intra-rachidial lumbar region, retroperitoneum and in bones. In the first case there was also involvement of the base of the cranium, which was responsible for the clinical symptoms of diabetes insipidus. In this case repeated biopsies and immunological studies suggested that disseminated fibrosclerosis might be related to Weber-Christian's disease.
D000328:Adult; D000368:Aged; D001847:Bone Diseases / Q000473:pathology*; D006332:Cardiomegaly / Q000209:etiology; D003919:Diabetes Insipidus / Q000150:complications; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009422:Nervous System Diseases / Q000473:pathology*; D010201:Panniculitis, Nodular Nonsuppurative / Q000473:pathology; D011658:Pulmonary Fibrosis / Q000150:complications; D012598:Sclerosis; D013256:Steroids / Q000627:therapeutic use
D013256:Steroids
Fibrosclérose multifocale. Trois cas avec atteinte du squelette et du système nerveux central.