Erdheim–Chester disease with cardiac involvement. Case report

Abstract

ErdheimChester disease (ECD) is a rare non-Langerhans histiocytosis with multisystem inflammatory infiltrates consistent of monocytes/macrophages, reactive microenvironment and fibrotic fields. Cardiovascular involvement is one of the most frequent manifestations of ECD that can lead to life threating complications. In this article we are reporting a clinical case of ECD with cardiac involvement in a young patient.

Keywords

• erdheim–chester disease
• non-langerhans cell histiocytosis
• mutation braf v600e
• trametinib