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

Lydia Giannitrapani, Maurizio Soresi, Aurelio Seidita, Giuseppe Brunori, Antonietta Serruto, Lydia Giannitrapani, Francesco Bencivinni, Maurizio Soresi, Antonietta Serruto, Aurelio Seidita, Francesco Bencivinni, Giuseppe Brunori

Risultato della ricerca: Articlepeer review


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.
Lingua originaleEnglish
pagine (da-a)64-70
Numero di pagine7
RivistaItalian Journal of Medicine
Stato di pubblicazionePublished - 2017

All Science Journal Classification (ASJC) codes

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