Rare presentation of multiple thromboses in Erdheim-Chester disease: a case-based review.

ABSTRACT:

Background

ECD is a rare non-Langerhans cell histiocytosis with diverse and heterogeneous clinical manifestations, ranging from single-lesion forms to multi-system involvement, including slowly progressing unifocal forms to rapidly evolving life-threatening disease.

Case presentation

A female patient presented with a 2-month history of fever. Imaging revealed multiple thromboses, bone destruction, an abnormal pituitary stalk, and clinical manifestations of diabetes insipidus. Excisional biopsy of a tibial lesion was sent for microscopic examination, and subsequent immunohistochemical testing was positive for expression of CD68 and CD163, and negative for expression of the immune markers CD1a, S100, and langerin. This confirmed the diagnosis of ECD. Treatment with methylpredn

SUBMITTER: Liu Y 

PROVIDER: S-EPMC9588466 | biostudies-literature | 2022 Nov

REPOSITORIES: biostudies-literature