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.

Clinical

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.

Treatment

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]

RegionalDerm

Homepage | FAQs | Use of Images | Contact Dr. White


It is not the intention of RegionalDerm.com to provide specific medical advice, diagnosis or treatment. RegionalDerm.com only intends to provide users with information regarding various medical conditions for educational purposes and will not provide specific medical advice. Information on RegionalDerm.com is not intended as a substitute for seeking medical treatment and you should always seek the advice of a qualified healthcare provider for diagnosis and for answers to your individual questions. Information contained on RegionalDerm.com should never cause you to disregard professional medical advice or delay seeking treatment. If you live in the United States and believe you are having a medical emergency call 911 immediately.