Langerhans cell histiocytosis in children: History, classification, pathobiology, clinical manifestations, and prognosis.

Abstract

Langerhans cell histiocytosis (LCH) is an inflammatory neoplasia of myeloid precursor cells driven by mutations in the mitogen-activated protein kinase pathway. When disease involves the skin, LCH most commonly presents as a seborrheic dermatitis or eczematous eruption on the scalp and trunk. Evaluation for involvement of other organ systems is essential, because 9 of 10 patients presenting with cutaneous disease also have multisystem involvement. Clinical manifestations range from isolated disease with spontaneous resolution to life-threatening multisystem disease. Prognosis depends on involvement of risk organs (liver, spleen, and bone marrow) at diagnosis, particularly on presence of organ dysfunction, and response to initial therapy. Systemic treatment incorporating steroids and cytostatic drugs for at least one year has improved prognosis of multisystem LCH and represents the current standard of care.

• Authors: Jolie Krooks, Milen Minkov, Angela G Weatherall
• Journal: Journal of the American Academy of Dermatology (J Am Acad Dermatol) Vol. 78 Issue 6 Pg. 1035-1044 (Jun 2018) ISSN: 1097-6787 [Electronic] United States
• PMID: 29754885 (Publication Type: Journal Article, Review)
• Copyright: Copyright © 2017 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

Topics

• Adolescent
• Biopsy, Needle
• Child
• Child, Preschool
• Disease Progression
• Female
• Histiocytosis, Langerhans-Cell (classification, epidemiology, pathology, therapy)
• Humans
• Immunohistochemistry
• Incidence
• Male
• Multimorbidity
• Prognosis
• Rare Diseases
• Risk Assessment
• Severity of Illness Index