Erdheim-Chester disease (ECD) is a rare, systemic, nonLangerhans cell histiocytosis. In approximately 25% of the cases there is cutaneous involvement. The diagnosis should be considered in patients with atypical or extensive xanthalasmata-like lesions with a normal lipid profile or systemic symptoms like bone pain or diabetes insipidus. ECD and Langerhans cell histiocytosis (LCH) can overlap (ECD-LCH overlap syndrome). Both seems to be associated with a BRAFV600E mutation, which suggests a common origin.

Additional Metadata
Keywords BRAFV600E mutation, Erdheim-Chester disease, Non-Langerhans cell histiocytosis
Persistent URL hdl.handle.net/1765/122833
Journal Nederlands Tijdschrift voor Dermatologie en Venereologie
Citation
Greveling, K, Zondag, T.E.C. (T. E.C.), Damman, J, van Laar, J.A.M, & van Doorn, M.B.A. (2019). De ziekte van erdheim-chester. Nederlands Tijdschrift voor Dermatologie en Venereologie, 29(5), 23–25. Retrieved from http://hdl.handle.net/1765/122833