Erdheim-Chester disease, an uncommon non-Langerhans cell histiocytosis and its association with Sjögren's syndrome

Abstract

Erdheim Chester disease (ECD) is a rare non-Langerhans cell histiocytosis. It is characterized by the infiltration of various organs and tissues by foamy histiocytes with a heterogeneous clinical course that varies from mild forms to disseminated forms with progressive and lethal behaviour. The case of a patient who presented with a cerebellar syndrome associated with autoimmune pathology is presented. In the course of the disease, refractoriness to glucocorticoids and clinical manifestation with bone involvement in the form of symmetrical osteosclerosis of long bones were determining factors for suspicion of this entity. We reviewed scientific articles through the PubMed metasearch engine with the keywords “erdheim chester disease”, “erdheim chester and nervous system”, and “autoimmunity and erdheim chester disease”, selecting those with greater emphasis on clinical presentation with neurological involvement and associated autoimmune pathology. Advances in the pathogenesis of ECD have allowed us to understand the nature of the disease, as well as the use of targeted therapies. It is interesting to keep this entity in mind, as well as the pathologies with which it is frequently associated, with the objective of an early diagnosis and a better clinical approach.