Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Erdheim-Chester disease: a case report.

Esther M Hoffmann1, Wibke Müller-Forell, Susanne Pitz

  • 1Department of Ophthalmology, University of Mainz, Langenbeckstr.1, 55131 Mainz, Germany. ehoffman@mail.uni-mainz.de

Graefe'S Archive for Clinical and Experimental Ophthalmology = Albrecht Von Graefes Archiv Fur Klinische Und Experimentelle Ophthalmologie
|June 29, 2004
PubMed
Summary

Erdheim-Chester disease (ECD) can affect the orbit, causing vision loss. This rare condition may mimic multifocal fibrosclerosis, highlighting the need for accurate diagnosis and tailored treatment strategies.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Machine Learning-Based Prediction of Long-Term Intraocular Pressure Fluctuations in Open-Angle Glaucoma.

Ophthalmology science·2026
Same author

Long-Term Patient Acceptability and Adherence to Suprachoroidal Telemetric Intraocular Pressure Monitoring in Glaucoma.

Journal of glaucoma·2026
Same author

Longitudinal Assessment of Large Optic Discs in Children and Adults Using Spectral Domain Optical Coherence Tomography.

Journal of clinical medicine·2026
Same author

PAUL® Glaucoma Implant versus Ahmed® Glaucoma Valve in Pediatric Glaucoma Following Congenital Cataract Surgery.

Journal of glaucoma·2026
Same author

The Association of Polygenic Risk Score With Glaucoma and Its Related Traits: Results From Two German Population-Based Studies.

Investigative ophthalmology & visual science·2026
Same author

Long-Term in vivo Positional Stability of a Suprachoroidal Telemetric Intraocular Pressure Sensor Implant.

Clinical ophthalmology (Auckland, N.Z.)·2025

Area of Science:

  • Histiocytosis and Inflammatory Diseases
  • Ophthalmology and Visual Science
  • Oncology and Hematology

Background:

  • Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized by xanthogranulomatous inflammation affecting multiple organs.
  • Orbital involvement in ECD, though rare, can lead to significant visual impairment, often presenting bilaterally.

Observation:

  • A 61-year-old male presented with bilateral exophthalmos and progressive vision loss due to retrobulbar pseudotumor.
  • Initial diagnosis considered multifocal fibrosclerosis based on retroperitoneal findings and orbital biopsy showing chronic fibrosing inflammation.
  • Despite radiotherapy and corticosteroids, orbital symptoms persisted, prompting further investigation.

Findings:

  • Re-evaluation of orbital biopsy revealed foamy cells and sclerotic changes, with positive CD68 staining, confirming Erdheim-Chester disease.

Related Experiment Videos

  • Immunohistochemistry (CD68+, S-100-, CD1a-) was crucial in differentiating ECD from other conditions.
  • The patient experienced a significant decline in visual acuity despite treatment, indicating a severe form of orbital ECD.

    • Implications:

    • Orbital ECD can manifest with severe, progressive vision loss unresponsive to standard treatments.
    • Clinical presentation of orbital ECD may overlap with multifocal fibrosclerosis, necessitating thorough diagnostic evaluation.
    • Accurate diagnosis of ECD is critical for appropriate management and to avoid therapeutic delays in patients with orbital involvement.