Medullary Lesions in Erdheim Chester Disease: A Case Report

Abstract

ERDHEIM CHESTER disease (ECD) called also non-Langerhans Histiocytosis, is a rare illness, which can present various unusual clinical aspects. It is a Histiocytosis characterized by an infiltration of various tissues and organs, bones, retroperitoneum, pleuro-pulmonary sites, skin, and central nervous system. The most frequent central nervous system manifestations of ECD are diabetes insipidus, cerebellar syndromes, orbital lesions, and extra-axial masses involving the dura and here we report a rare case with spinal cord compression on the context of ERDHEIM CHESTER disease. Our work highlights another different clinical, radiological and pathological manifestation associated with ECD that should be considered.