Eosinophilic Granuloma, Bilateral Mastoid Involvement

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IN review of the literature it was found that xanthomatous lesions of the temporal bones are not unusual, but that only 3 cases of bilateral mastoid involvement have been reported1,2,3 since Schuller and Christian discussed, in 1915 and 1919 respectively, cases presenting a syndrome now known as Hand-Schuller-Christian’s disease. Hand’s name became associated with this syndrome because he reported one case in 1893 and another in 1921 which presented the clinical triad of diabetes insipidus, exophthalmos, and bony defects of the skull — the findings necessary to establish the diagnosis of Hand-Schuller-Christian’s disease. He attributed this to tuberculosis, however.

In 1932 Creifenstein4 reviewed the 26 cases of Hand-Schuller-Christian’s disease appearing in the literature up to that time and noted that the ear was involved in 12 of these cases. Not until 1935 did an abundance of literature on Hand-Schuller-Christian’s disease or eosinophilic granuloma appear in the journals; several years later the true relationship between these two entities and Abt-Letterer-Siwe’s disease was established.

The histopathology involved in this syndrome was defined in 1928 by Rowland5 who concluded that it consisted of a form of xanthoma involving the reticulo-endothelial system of certain areas of the skull. However, it was not until 1940 to 1948 that the close association of Abt-Letterer-Siwe’s disease, Hand-Schuller-Christian’s disease and eosinophilic granuloma, as variants of the same xanthomatous disease, was recognized.6

In view of the infrequent instances of bilateral mastoid involvement by eosinophilic granuloma found in the literature, it was felt that this case would provide an interesting report.



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