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

Autor:	Akiki Béatrice, Aftimos Georges, Makarem Jawad, Rémie Philippe Elia, Zeidan Marwan, Waked Hani, Atallah Adnan
Rok vydání:	2020
Předmět:	Pathology medicine.medical_specialty integumentary system Langerin biology business.industry General Medicine medicine.disease BRAF V600E Histiocytosis Immune system Bone lesion Histiocytic proliferation Erdheim–Chester disease medicine biology.protein business
Zdroj:	Case Reports in Clinical Pathology. 7:22
ISSN:	2331-2734 2331-2726
DOI:	10.5430/crcp.v7n1p22
Popis:	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.
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=doi_________::0e0db93417c037f104eb15797f6efacb https://doi.org/10.5430/crcp.v7n1p22 Zobrazit plný text záznamu