ACUTE IDIOPATHIC CHOLESTEROL GRANULOMATOSIS

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.