Eosinophilic Granuloma (80%) Localized benign form Isolated to bone Hand-Schuller-Christian disease (15-20%) Skull lesions Exopthalmos Diabetes Insipidus Letterer-Siwe disease (>10%) Disseminated lesions involving multiple visceral organs Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the presence of cells with characteristics similar to bone marrow–derived Langerhans cells juxtaposed against a backdrop of hematopoietic cells, including T-cells, Reticulum cells The bones which are the most commonly involved are the skull, the pelvis, and the diaphysis of long bones. The systemic condition includes Hand-Schüller-...

Important points for the paediatrician and Paediatric radiologist regarding bone LCH include the following: _ LCH should be considered in all young patients whopresent with a skull mass, jaw pain, swelling and/or loose teeth, chronic ear drainage, with dermatitis of the auricular canal, mastoiditis and cholesteatoma or proptosis, swelling and redness of the eyelid. The classical radiologic finding is a punched-out lytic lesion in bone, but some LCH lesions can resemble an aggressive bone sarcoma with destruction of bone and periosteal elevation [40]. This is...

This exhibit illustrates the spectrum of Imagng features of LCH in its typical atypical location

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