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Related Experiment Video

Updated: Jul 14, 2026

Three-Dimensional Reconstruction of Orbital Fractures

Three-Dimensional Reconstruction of Orbital Fractures

Published on: May 16, 2025

Orbital involvement in Erdheim-Chester disease.

Winnie W Y Lau¹, Edwin Chan, Clement W N Chan

¹Department of Ophthalmology, Queen Mary Hospital, Pokfulam Road, Hong Kong. lauwaiyingwinnie@gmail.com

Hong Kong Medical Journal = Xianggang Yi Xue Za Zhi

|June 6, 2007

Summary

Erdheim-Chester disease, a rare histiocytic disorder, can present with unusual orbital symptoms. Early recognition is crucial for managing this potentially fatal condition, especially in underrepresented populations.

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Last Updated: Jul 14, 2026

Three-Dimensional Reconstruction of Orbital Fractures

Three-Dimensional Reconstruction of Orbital Fractures

Published on: May 16, 2025

Area of Science:

Histiocytic Disorders
Rare Diseases
Oncology

Background:

Erdheim-Chester disease (ECD) is a rare, idiopathic, non-Langerhans' cell histiocytic disorder.
This report details the second known case of ECD in the Chinese population.

Observation:

A 45-year-old woman presented with unilateral proptosis and periorbital xanthelasma.
Histopathology showed a xanthogranulomatous lesion (CD68+, S100-, CD1a-, CD3-, CD20-).
Bone scan revealed systemic involvement; CT showed thoracic and abdominal aorta involvement.

Findings:

Despite aggressive treatment (steroids, immunosuppressants, chemotherapy, interferon), the patient experienced progressive deterioration.
The clinical course aligned with existing literature on Erdheim-Chester disease.
The patient succumbed to neutropenic fever and septic shock.

Implications:

Unusual presentations of ECD, particularly orbital masses and xanthelasma, warrant high suspicion.
Prompt diagnosis and management are critical for improving outcomes in this rare and severe disease.
This case highlights the importance of considering ECD in diverse ethnic groups.