Erdheim-Chester disease: a case report

Graefe's Archive for Clinical and Experimental Ophthalmology = Albrecht Von Graefes Archiv Für Klinische Und Experimentelle Ophthalmologie
Esther M HoffmannHerbert Radner

Abstract

Erdheim-Chester disease (ECD) is a rare histiocytosis, histologically characterized by xanthogranulomatous inflammation. It may affect the bones, heart, lung, liver, kidneys, retroperitoneum, brain, and, rarely, the orbit. In the majority of patients, orbital infiltration is bilateral. A 61-year-old man presented with bilateral exophthalmos and progressive loss of visual function caused by chorioretinal folds and papillary swelling due to retrobulbar pseudotumor. The computed tomogram of the abdomen showed retroperitoneal thickening, leading to the interpretation of multifocal fibrosclerosis. This was further substantiated by an orbital biopsy revealing chronic fibrosing inflammation. Retrobulbar radiotherapy (20 Gy) and long-term systemic corticosteroid treatment followed. Although the retroperitoneal involvement decreased, no significant effect on orbital involvement was achieved. A second review of the orbital biopsy revealed foamy cell infiltration and the presence of a sclerotic process. Immunohistochemical examination demonstrated positive CD 68 stains, whereas S-100 and CD 1a were negative, thus confirming ECD. The histologic finding was comparable to a biopsy of the retroperitoneum. Endonasal decompression was performed...Continue Reading

References

Nov 21, 1997·Archives of Ophthalmology·C ValmaggiaI Gottlob
Feb 5, 1999·European Radiology·W KennG Wittenberg
Sep 4, 1999·The Netherlands Journal of Medicine·I van der LeeJ R Elbers
Oct 21, 2000·Eye·T G SheidowJ G Heathcote
Jun 29, 2001·Der Hautarzt; Zeitschrift für Dermatologie, Venerologie, und verwandte Gebiete·D F WatermannP J Frosch
Sep 25, 2001·The British Journal of Ophthalmology·J R Smith, J T Rosenbaum
Jun 6, 2002·Orbit·Paolo de PalmaIngrid Nielsen
Jun 18, 2002·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Samer Al-QuranLisa Rimsza
Jul 31, 2002·Experimental and Clinical Endocrinology & Diabetes : Official Journal, German Society of Endocrinology [and] German Diabetes Association·B PerrasH L Fehm
Aug 21, 2002·The American Journal of the Medical Sciences·Anu GuptaJames E McGuigan
Sep 21, 2002·Journal of Endocrinological Investigation·M E KhamsehF Azizi

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Citations

Sep 25, 2009·Der Ophthalmologe : Zeitschrift der Deutschen Ophthalmologischen Gesellschaft·K ManousaridisR F Guthoff
Oct 24, 2006·Transactions of the American Ophthalmological Society·Victor M ElnerAdam S Hassan
Mar 27, 2010·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Alexandria ConleySandeep Mittal
Aug 3, 2012·Veterinary Ophthalmology·Stephanie A PumphreyFaith I Buckley
Sep 9, 2006·Radiotherapy and Oncology : Journal of the European Society for Therapeutic Radiology and Oncology·Robert C MillerTimothy G Call
Dec 20, 2008·Journal of Korean Neurosurgical Society·Jae-Hyun KwonKi-Uk Kim
May 27, 2010·Ophthalmic Plastic and Reconstructive Surgery·Katayoon B EbrahimiW Richard Green
May 9, 2014·Ophthalmic Plastic and Reconstructive Surgery·Heather M WeissmanHans E Grossniklaus
Apr 20, 2014·Journal of Neuro-ophthalmology : the Official Journal of the North American Neuro-Ophthalmology Society·William L HillsMark L Moster
Jan 19, 2006·Ophthalmic Plastic and Reconstructive Surgery·Victor M ElnerAdam S Hassan
Aug 13, 2015·International Cancer Conference Journal·R Chicas SettA Tormo Micó
Jun 4, 2021·Survey of Ophthalmology·Menelaos KanakisIlias Georgalas

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