Rare diseases of bone: Erdheim-Chester and Rosai-Dorfman non-Langerhans cell histiocytoses

Andreas F Mavrogenis; Vasilios G Igoumenou; Thekla Antoniadou; Panayiotis D Megaloikonomos; George Agrogiannis; Periklis Foukas; Sotirios G Papageorgiou

doi:10.1302/2058-5241.3.170047

Rare diseases of bone: Erdheim-Chester and Rosai-Dorfman non-Langerhans cell histiocytoses

EFORT Open Rev. 2018 Jun 23;3(6):381-390. doi: 10.1302/2058-5241.3.170047. eCollection 2018 Jun.

Authors

Andreas F Mavrogenis¹, Vasilios G Igoumenou¹, Thekla Antoniadou¹, Panayiotis D Megaloikonomos¹, George Agrogiannis², Periklis Foukas², Sotirios G Papageorgiou³

Affiliations

¹ First Department of Orthopaedics, National and Kapodistrian University of Athens, School of Medicine, Greece.
² Second Department of Pathology, National and Kapodistrian University of Athens, School of Medicine, Greece.
³ Second Department of Internal Medicine, Hematology Unit, National and Kapodistrian University of Athens, School of Medicine, Greece.

Abstract

Non-Langerhans cell histiocytosis (N-LCH) summarizes a group of rare diseases with different clinical presentations, pathogenesis and morphology. These include primary cutaneous N-LCH, cutaneous N-LCH with systemic involvement, and primary extracutaneous systemic forms with occasional cutaneous involvement.The juvenile (JXG) and non-juvenile xanthogranuloma (N-JXG) family of histiocytoses are N-LCH: the JXG family consisting of the JXG (cutaneous), xanthoma disseminatum (cutaneous and systemic) and Erdheim-Chester disease (ECD; systemic); and the N-JXG family consisting of the solitary reticulohistiocytoma (cutaneous), multicentric reticulohistiocytosis (cutaneous and systemic) and Rosai-Dorfman disease (RDD; systemic).ECD is a clonal disorder from the JXG family of N-LCH; RDD is a reactive proliferative entity from the non-juvenile xanthogranuloma family of N-LCH.ECD and RDD N-LCH are rare disorders, which are difficult to diagnose, with multi-organ involvement including bone and systemic symptoms, and which respond to therapy in an unpredictable way.The key to successful therapy is accurate identification at tissue level and appropriate staging. Patients should be observed and monitored in a long-term pattern. Prognosis depends on disease extent and the organs involved; it is generally good for RDD disease and variable for ECD. Cite this article: EFORT Open Rev 2018;3:381-390. DOI: 10.1302/2058-5241.3.170047.

Keywords: Erdheim-Chester disease; Rosai-Dorfman disease; bone; juvenile xanthogranuloma; non-Langerhans cell histiocytosis; non-juvenile xanthogranuloma.