Craniofacial and intracranial manifestations of langerhans cell histiocytosis: report of findings in 100 patients.

Abstract

The purpose of this study was to review the craniofacial and intracranial clinical and radiologic manifestations of patients diagnosed with Langerhans cell histiocytosis (LCH). This report will compare the frequency of the various manifestations found in our series with those reported in the medical literature. In LCH, involvement of the calvaria, skull base, maxillofacial bones, and hypothalamic-pituitary axis is fairly common. The precise location of these lesions contributes to the variety of clinical manifestations of LCH, which includes scalp and/or facial swelling, seizures, hearing loss, recurrent otitis media, gingival bleeding, proptosis, diabetes insipidus, and cranial nerve palsies.