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Medicine in Images
Corneal involvement due to Erdheim-Chester disease
Afectación corneal debido a enfermedad de Erdheim-Chester
J. Díez de los Ríosa,
Corresponding author
jdiez@chv.cat

Corresponding author.
, J. Serra Batllesb, F. Ruizb
a Servicio de Medicina Interna, Hospital Universitario de Vic, Vic, Barcelona, Spain
b Servicio de Neumología, Hospital Universitario de Vic, Vic, Barcelona, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We present the case of a 50-year-old man with a medical history of Erdheim-Chester disease diagnosed in 1992&#44; with multiple organ involvement &#40;corneal&#44; skin&#44; neurological&#44; bone and vascular&#41;&#44; who was hospitalized for symptoms of dry cough and dyspnea with acute respiratory failure&#46; The patient was diagnosed with interstitial lung disease&#46; The physical examination revealed corneal infiltration by histiocytes &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#44; previously treated with corneal transplantation&#44; with subsequent graft rejection&#44; resulting in blindness&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">After being discharged and due to ocular pain&#44; the patient underwent surgery to enucleate both eyes&#46; The biopsy of the right globe showed non-necrotizing xanthogranulomatous inflammation affecting the cornea and focally affecting the sclera&#44; with obliteration of the anterior chamber&#46; The biopsy of the left globe showed non-necrotizing xanthogranulomatous inflammation affecting the sclera and the irido-sclero-corneal angle&#46; The morphological changes were consistent with Erdheim-Chester disease&#44; a rare histiocytosis of non-Langerhans cells characterized by multisystem infiltration by epithelioid cells and foamy histiocytes&#46; Approximately 700 cases have been described in the literature&#46; Intraocular manifestations are extremely rare&#44; with 5 published cases &#40;4 with choroid infiltration and one with subretinal infiltration&#41;&#46; Our case exceptionally presented corneal involvement&#44; which has not been previously reported&#46;</p></span>"
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Original language: English
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