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https://doi.org/10.14300/mnnc.2024.19079

Erdheim – Chester histiocytosis. Clinical observation

[Notes from practice]
Alexandr Yagoda; Stepan Sergeevich Oplochko; Natalia Gladkikh; Pavel Koroy; Anna Sergeevna Bataeva;

Erdheim – Chester disease (ECD) belongs to rare types of pathology classified as histiocytic clonal disease, which is included in the «tumors of histiocytes and dendritic cells» category. The issue is diagnosed based on specific X-ray scintigraphic image depicting the skeleton lesions; tissue histological examination identifying foamy histiocytosis; inflammation signs, as well as giant Touton cells. This paper features a clinical case of a two-year-long course of ECD in a patient where the respective damage involves the skeleton bones, the nervous system, with initial manifestations of retroperitoneal infiltration.

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Keywords: Erdheim – Chester disease, histologically verified, damage to the skeleton, nervous system, Horner syndrome,diplopia, diagnostic difficulties