Eosinophilic Granuloma

This male patient presented at age 3 with a mandibular mass. Biopsy showed a dense infiltrate of cells with eosinophilic cytoplasm and grooved nuclei, typical of Langerhan’s cells. There were also numerous eosinophils. A subsequent skeletal survey demonstrated no other lesions. Two years later, a repeat skeletal survey shows characteristic lesions in femur, ribs and skull.
The lateral skull xray shows a characteristic lesion in the parietal bone, with the bevelled-edge sign (two distinct margins can be appreciated, representing the inner and outer tables).

Langerhan’s cell histiocytosis is a heterogeneous group of disorders, including Letterer-Siwe disease (disseminated fulminant disease, typically infants); Hand-Schuller-Christian disease (chronic disseminated disease, typically children); and eosinophilic granuloma of bone (70% of cases).

The characteristic appearance of the skull in Hand-Schuller-Christian disease was likened (by Schuller) to a map РLandkartenschädel.

Eosinophilic granuloma of bone is polyostotic in 25-50% of cases, which does not imply dissemination beyond bone. Disseminated forms of LCH may involve lymph nodes, liver, spleen, thymus, gastrointestinal tract and endocrine system. The prognosis for disease limited to bone is good. Disseminated disease is more aggressive, with 15% mortality in Hand-Schuller-Christian, and 70% mortality in Letterer-Siwe.

Reference: Dähnert W. Radiology Review Manual, 5th edition, Lippincott, Williams & Wilkins, 2003.

Credit: Dr Laughlin Dawes