Erdheim-Chester Disease: A Case Report

The diagnosis of Erdheim-Chester disease, a rare illness, is difficult and requires increased awareness.

We report the case of a 56-year-old woman who initially presented with a mesenteric panniculitis and 8 years later developed neurological manifestations and bone lesions that led to a diagnosis of Erdheim-Chester disease.

The rather characteristic aspect of the bone lesions as well as the presence of foamy cells in involved tissue biopsies should suggest the diagnosis. No therapy is available at present but recent biological data might suggest new approaches for the understanding and therapy of this condition.