Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2025 Sep;17(13):913-916.
doi: 10.1080/1750743X.2025.2561393. Epub 2025 Sep 17.

Multifocal sclerotic lesions in Erdheim-Chester disease successfully treated with tocilizumab

Henk-Jan Boiten  1 , E J Libourel  1
Affiliations
Case Reports

Multifocal sclerotic lesions in Erdheim-Chester disease successfully treated with tocilizumab

Henk-Jan Boiten et al. Immunotherapy. 2025 Sep.

Abstract

A 58-year-old woman presented to the outpatient clinic with invalidating bone pain in the proximal lower extremities. Laboratory testing showed increased inflammation parameters with a normocytic anemia. Bone scintigraphy revealed increased uptake in both radii, distal femurs, tibiae, and fibulae. PET/CT scanning showed irregular sclerotic bone lesions without other abnormalities. Multiple bone biopsies showed histiocytes expressing CD68 and Factor XIIIa. Metastatic or hematopoietic neoplasms were ruled out. A diagnosis of Erdheim-Chester disease (ECD) with solely osseous involvement was made. Treatment with tocilizumab weekly, an IL-6 receptor antibody, was successful. Tocilizumab appears to be a promising agent for the treatment of patients with Erdheim-Chester disease with limited organ involvement.

Keywords: Erdheim–Chester disease; IL-6; bone involvement; histiocytosis; tocilizumab.

PubMed Disclaimer

Conflict of interest statement

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

No writing assistance was utilized in the production of this manuscript.

References

    1. Younes IE, Ellis A, Zhang X.. Updates on Erdheim-Chester disease. Hum Pathol Rep. 2022;28:300636. doi: 10.1016/j.hpr.2022.300636 - DOI
    1. Goyal G, Heaney ML, Collin M, et al. Erdheim-Chester disease: consensus recommendations for evaluation, diagnosis, and treatment in the molecular era. Blood. 2020;135(22):1929–1945. doi: 10.1182/blood.2019003507 - DOI - PubMed

    2. • The latest guidelines concerning the diagnostic workup and therapeutic management of patients with Erdheim-Chester disease.

    1. Emile JF, Abla O, Fraitag S, et al. Histiocyte society. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood. 2016;127(22):2672–2681. doi: 10.1182/blood-2016-01-690636 - DOI - PMC - PubMed
    1. Cohen-Aubart F, Emile JF, Carrat F, et al. Phenotypes and survival in Erdheim-Chester disease: results from a 165-patient cohort. Am J Hematol. 2018;93(5):E114–E117. doi: 10.1002/ajh.25055 - DOI - PubMed
    1. Milne P, Bigley V, Bacon CM, et al. Hematopoietic origin of Langerhans cell histiocytosis and Erdheim-Chester disease in adults. Blood. 2017;130(2):167. doi: 10.1182/blood-2016-12-757823 - DOI - PMC - PubMed

Publication types

MeSH terms

LinkOut - more resources