Cervical Lymphadenopathies as Unusual Presentations of Erdheim-Chester Disease: The Need for Knowledge for Diagnosis and Treatment.

The appearance of cervical adenopathies can occur in many pathologies in a non-specific manner; Erdheim-Chester disease (ECD) is characterized by xanthogranulomatous and xanthomatous infiltration of different tissues with numerous foamy histiocytes. Bone lesions are frequent and radiological features are pathognomonic for diagnosis, but lymph node involvement is exceptional and is not a form of presentation reported in the literature. Recurrent BRAFV600E mutation and others have been discovered in recent years.