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Article
July 1965

Hand-Schüller-Christian Disease With Occult Diabetes Insipidus

Author Affiliations

BALTIMORE

From the Gerontology Branch, National Heart Institute, Public Health Service, US Department of Health, Education, and Welfare, Bethesda, the departments of medicine and pathology, Baltimore City Hospitals, and the Johns Hopkins University School of Medicine, Baltimore. Medical Officer, US Public Health Service, Gerontology Branch, National Heart Institute, Baltimore City Hospitals and Instructor in Medicine, Johns Hopkins University School of Medicine (Dr. Lindeman); Resident in Pathology, Baltimore City Hospitals (Dr. Sear); and Medical Officer, US Public Health Service, Gerontology Branch, National Heart Institute, Baltimore City Hospitals and Assistant in Medicine, Johns Hopkins University School of Medicine (Dr. Lee).

Arch Intern Med. 1965;116(1):131-137. doi:10.1001/archinte.1965.03870010133017
Abstract

THE CLASSIFICATION of a granulomatous lesion showing reticuloendothelial hyperplasia, histiocytes with clear lipid deposits, and giant cells engulfing white and red blood cells is often dependent upon a knowledge of the clinical course of the host. Closely related histologically but with different clinical pictures are the isolated benign eosinophilic granuloma, the chronic disseminated Hand-Schiiller-Christian disease, and the generalized, usually rapidly fatal, Letterer-Siwe disease. Individuals with these granulomatous lesions described in the initial reports of Hand,1 Schuller2 and Christian 3 had a common triad of findings consisting of osteolytic defects of membranous bone (skull), diabetes insipidus, and exophthalmos. Subsequent observations on similarly afflicted individuals indicate these findings may be observed singly or in combination with additional involvement of other bones, lymph nodes, lung, skin, central nervous system, liver, spleen, and rarely other tissues.

The 88-year-old Negro woman reported herein with evidence of chronic multiple system involvement (bone, lymph

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