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Case Reports
October 1941


Author Affiliations

From the Department of Pediatrics of New York Medical College, Flower and Fifth Avenue Hospitals, and the Department of Pathology of the Metropolitan Hospital, Department of Hospitals, New York City.

Am J Dis Child. 1941;62(4):776-792. doi:10.1001/archpedi.1941.02000160077009

In 1893 Hand1 described the "peculiar case" of a 3 year old boy with "tuberculosis and polyuria"; the disorder was later recognized as idiopathic xanthomatosis. In 1915 Schüller,2 in roentgen studies of the bones, noted osseous defects, especially common in the skull, associated with exophthalmos and diabetes insipidus. Christian3 in 1919 gave the classic description of the disease, which he regarded as resulting from endocrine glandular dysfunction. In 1923 Eppinger4 expressed the opinion that this disease occurred as a result of disturbance of lipoid metabolism, a theory which was later confirmed chemically by Epstein and Lorenz.5 Rowland6 was the first to express the idea that Schüller-Christian disease is a primary disturbance of lipoid metabolism leading to secondary proliferation of the reticuloendothelial cells. This opinion has been widely accepted.7

Letterer8 in 1924 described the case of a 6 month old child who had reticuloendotheliosis with little lipoid storage.

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