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Xanthogranuloma of the dura in systemic Weber-Christian disease

Department of Pathology (Neuropathology), Rose F. Kennedy Center for Mental Retardation and Human Development, Albert Einstein College of Medicine, and Bronx Municipal Hospital Center, Bronx, NY.

A 51-year-old woman who had systemic Weber-Christian disease (WCD) displayed multiple xanthomatous lesions and a large xanthogranuloma of the tentorium cerebelli with signs of brainstem and cerebellar compression. Three other cases of dural xanthomatous lesions associated with systemic WCD have been reported. These dural and leptomeningeal xanthomatous lesions in WCD, a nonsuppurative inflammatory condition, are similar to those of Hand-Schüller-Christian disease, a quasi-neoplastic process.

Address correspondence and reprint requests to Dr. Horoupian, Department of Pathology (Neuropathology), Rose F. Kennedy Center, K-438, Albert Einstein College of Medicine, 1300 Morris Park Avenue, Bronx, NY 10461.

Accepted for publication December 4, 1982.