Intracranial xanthogranuloma of the dura in Hand-Schüller-Christian disease

Abstract

The authors describe a 37-year-old man with the classic clinical features of Hand-Schüller-Christian disease. He presented with symptoms of increased intracranial pressure due to obstructive hydrocephalus secondary to a huge xanthogranuloma involving falx cerebri and tentorium cerebelli. Immunohistochemical and ultrastructural studies failed to demonstrate Langerhans histiocytes, however. The implication of this finding is discussed in light of the recent relevant literature.