Erdheim Chester disease: a subtle quiddity; the first case reported from Nepal

Abstract

Erdheim-Chester disease (ECD) is a rare entity throughout the world. This is the first case reported in Nepal. ECD is a rare aggressive, non-Langerhan's histocytosis of unknown origin with classical histological features. The patient usually presents with bone pain or skeletal symptoms along with other constitutional syndrome. Although, no definitive therapy has been approved, interferon-alfa (or Pegylated Interferon-alfa) is considered as initial therapy. In this case report, we found a patient with right-sided localized chest pain for which he was evaluated with bone scan and excisional bone biopsy and its findings support the diagnosis of ECD.

Keywords: CT, Computed Tomography; ECD, Erdheim-Chester Disease; Erdheim-Chester disease; Interferon-alfa; MDP, Methylene Diphosphonate; Nepal; Non-Langerhan's histocytosis; SPECT, Single-Photon Emission Computed Tomography.