JOURNAL ARTICLE

O03 Langerhans cell histiocytosis: case series of presentations to dermatology in the BRAF era.

Published In: British Journal of Dermatology, 2023, v. 189, n. 3. P. e41 1 of 3
Database: Academic Search Ultimate 2 of 3
Authored By: Coyle, Conor; Bovijn, Louise; Blanco, Esther; McPherson, Tess 3 of 3

Abstract

This article focuses on Langerhans cell histiocytosis (LCH), a rare disease driven by MAPK pathway mutations, highlighting its clinical heterogeneity and the role of dermatology in diagnosis and management. A review of 14 pediatric patients seen between 2015 and 2022 showed varied cutaneous manifestations and a predominance of multisystem disease, particularly among those with the BRAF V600E mutation. Patients with this mutation exhibited more severe multisystem involvement, including diabetes insipidus and adrenal insufficiency, and some responded well to targeted therapy with dabrafenib. The findings emphasize the need for further research on the relationship between genetic alterations, clinical phenotypes, and comorbidities in LCH.

Additional Information

Source:British Journal of Dermatology. 2023/09, Vol. 189, Issue 3, pe41
Document Type:Article
Subject Area:Health and Medicine
Publication Date:2023
ISSN:0007-0963
DOI:10.1093/bjd/ljad259.003
Accession Number:172425492
Copyright Statement:Copyright of British Journal of Dermatology is the property of Oxford University Press / USA and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

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