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Cutaneous Langerhans cell histiocytosis

Cutaneous Langerhans cell histiocytosis Langerhans cell histiocytosis (LCH) is a rare disorder that is now recognized as a true malignancy of dendritic Langerhans cells. Various somatic mutations have been discovered for BRAF and MAP kinases. The clinical variability is remarkable. A very rare variant is cutaneous LCH without internal organ manifestations. We present a case series of cutaneous LCH illustrating the spectrum of clinical symptoms, treatment, and disease course. Cutaneous LCH needs a careful investigation of possible internal involvement and a long follow-up since second malignancies may develop over time. Overtreatment should be avoided, since too-aggressive treatment will not improve the outcome. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Wiener Medizinische Wochenschrift Springer Journals

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Publisher
Springer Journals
Copyright
Copyright © 2016 by Springer-Verlag Wien
Subject
Medicine & Public Health; Medicine/Public Health, general; Pharmacology/Toxicology; General Practice / Family Medicine; Geriatrics/Gerontology; Internal Medicine; Infectious Diseases
ISSN
0043-5341
eISSN
1563-258X
DOI
10.1007/s10354-016-0460-3
Publisher site
See Article on Publisher Site

Abstract

Langerhans cell histiocytosis (LCH) is a rare disorder that is now recognized as a true malignancy of dendritic Langerhans cells. Various somatic mutations have been discovered for BRAF and MAP kinases. The clinical variability is remarkable. A very rare variant is cutaneous LCH without internal organ manifestations. We present a case series of cutaneous LCH illustrating the spectrum of clinical symptoms, treatment, and disease course. Cutaneous LCH needs a careful investigation of possible internal involvement and a long follow-up since second malignancies may develop over time. Overtreatment should be avoided, since too-aggressive treatment will not improve the outcome.

Journal

Wiener Medizinische WochenschriftSpringer Journals

Published: Jul 5, 2016

References