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Klin Monbl Augenheilkd 2025; 242(11): 1086-1089
DOI: 10.1055/a-2405-7040
Der interessante Fall

Case Report: Periocular Steroid Injection Combined Vemurafenib Therapy in the Long-term Management of Orbital Involvement of Erdheim-Chester Disease

Fallbericht: periokulare Steroidinjektion, kombinierte Vemurafenib-Therapie auf lange Sicht beim Management der orbitalen Beteiligung der Erdheim-Chester-Krankheit

Authors

  • Fatma Selin Kaya

    1   Ophthalmology Department, Başakşehir Çam and Sakura City Hospital, Basaksehir/Istanbul, Turkey

  • Hatem Hakan Selçuk

    2   Radiology Department, Istanbul Acibadem Hospital, Atasehir, Istanbul, Turkey

  • Seyhan Ordekçi

    3   Microbiology Department, Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training, Research Hospital, Istanbul, Turkey

  • Bengi Demirayak

    4   Ophthalmology Department, Istanbul Bakirköy Dr. Sadi Konuk Research and Education Hospital Bakirköy, Istanbul, Turkey

  • Sibel Zirtiloglu 

    5   Ophthalmology Department, Kanuni Sultan Süleyman Training and Research Hospital, Zentinburnu, Turkey

The authors declare that they have not received grant support or research funding, and they do not have any proprietary interests in the materials described in the article.
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Introduction

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized by systemic xanthogranulomatous infiltration [1]. Ophthalmologic involvement occurs in 25 – 30% of ECD cases, usually in the form of orbital involvement presenting with exophthalmos and ophthalmoplegia [2]. ECD with orbital involvement is a devastating disease with a poor prognosis. A review of the literature and past experiences suggest that ECD often follows an aggressive course despite treatment. The literature reports a large range of treatment modalities that have been used to manage patients with ECD [3]. Vemurafenib has also been tried, with a recent report of a good response and clinical improvement in ECD eye involvement [4]. Here, we report the case of a patient who presented with progressive visual loss 2 years after diagnosis of ECD and long-term follow-up with vemurafenib therapy.

The study followed the tenets of the Declaration of Helsinki. The patient signed a written informed consent for the research use of clinical records and data included in the study.



Publication History

Received: 14 March 2024

Accepted: 26 August 2024

Accepted Manuscript online:
29 August 2024

Article published online:
14 November 2024

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