An unusual Erdheim-Chester disease with orbital involvement: a case report

  • Giuseppe Brunori | eko83m@libero.it Biomedical Department of Internal Medicine, University Hospital of Palermo, Italy.
  • Aurelio Seidita Biomedical Department of Internal Medicine, University Hospital of Palermo, Italy.
  • Lydia Giannitrapani Biomedical Department of Internal Medicine, University Hospital of Palermo, Italy.
  • Antonietta Serruto Biomedical Department of Internal Medicine, University Hospital of Palermo, Italy.
  • Francesco Bencivinni Centralized Imaging Diagnostics Service, University Hospital of Palermo, Italy.
  • Maurizio Soresi Biomedical Department of Internal Medicine, University Hospital of Palermo, Italy.

Abstract

Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis with multiorgan involvement and a specific tropism for perivascular and fatty connective tissue, of unclear origin, with poor response to therapy. Its identification is difficult because of the variable clinical presentation and its lack of knowledge. We report the case of a 63-years-old woman, with a history of bilateral orbital pseudotumor, who comes to our attention because of progressively worsening asthenia, vomiting and systemic inflammation. Total body computerized tomography scan showed a volumetric increase of choroid plexus of the temporal horn of the left lateral ventricle, presence of solid retrobulbar tissue at the level of both maxillary sinuses, lung fibrosis, and retroperitoneal and peri-aortic infiltration. The association of these signs addressed to a diagnosis of Erdheim-Chester disease. Thus, although extremely rare, the diagnosis of Erdheim-Chester disease must be considered in the case of bilateral retro-orbital tumors and multisystemic involvement.

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Published
2016-11-29
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Section
Case Reports
Keywords:
Erdheim-Chester disease, non-Langerhans cell histiocytosis, bilateral retro-orbital tumors, multisystemic involvement.
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How to Cite
Brunori, G., Seidita, A., Giannitrapani, L., Serruto, A., Bencivinni, F., & Soresi, M. (2016). An unusual Erdheim-Chester disease with orbital involvement: a case report. Italian Journal of Medicine, 11(1), 64-70. https://doi.org/10.4081/itjm.2016.700