LANGERHANS CELL HISTIOCYTOSIS-ADULTS
By Gary M. White, MD
Langerhans Cell Histiocytosis (LCH) is an abnormal proliferation of cells that resemble normal Langerhans cells. A high number of BRAF V600E mutations have been detected.
Adults who develop LCH may present with multiple, disseminated, discrete yellow-red papules often in the body folds. The vulva may be involved [JAMADerm March 2014;326] with destruction of the normal architecture. Workup should include systemic evaluation with a focus on the bones, reticuloendothelial system, lungs, orbit, and hypothalamic-pituitary axis.
See, for example, Orphanet J Rare Dis. 2013 May 14;8:72. One patient with a BRAF V600E mutation had a major response to vemurafenib [JAAD 2014;71;e97]
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