Erdheim chester disease: A case report and review of the literature

Rémie Philippe Elia, ATALLAH Adnan, AKIKI Béatrice, WAKED Hani, ZEIDAN Marwan, MAKAREM Jawad, AFTIMOS Georges


Erdheim Chester disease is a rare form of non-Langerhans histiocytosis with frequent BRAF V600E mutations. It is mainly characterized by multifocal osteosclerotic bone lesions with or without systemic involvement. The histologic image is consistent with a histiocytic proliferation of foamy cells in a polymorphic background. The main difference from the Langerhans histiocytosis is the immune profile with mainly S100, CD1a, and langerin negative. The overall prognosis is dependent on extraskeletal involvement. Herein, we present a typical presentation of Erdheim Chester disease with a review of the literature.

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Case Reports in Clinical Pathology

ISSN 2331-2726(Print)  ISSN 2331-2734(Online)

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