Diverse Cutaneous Presentations of Langerhans Cell Histiocytosis in Children: A Retrospective Cohort Study.

Abstract:

BACKGROUND:Langerhans cell histiocytosis (LCH) is a rare disease, frequently affecting young children. PROCEDURE:We performed a retrospective study in patients younger than 16 years old manifesting with skin symptoms, and documented their different cutaneous lesions and systemic symptoms. We compared subgroups of children with single-system, skin-only, and multisystem disease and sought signs predictive for multisystem disease. In a small sample of patients, BRAF mutations were analyzed in archived biopsies. RESULTS:A wide spectrum of cutaneous presentations varying from crusted nodules and papules, blisters, vascular tumor-like lesions, scaling orange to red macules (frequently in seborrheic regions) to purpuric macules, and papules was documented in our cohort of 32 children. Otitis externa was a common manifestation and mucosal lesions were seen in three patients. A novel manifestation was a red-blue nodule that appeared in a patient after a vaccination. None of the cutaneous lesions was predictive for the classification or final outcome as a single-system or multisystem disease. However, later onset and a more protracted course of skin lesions were more frequent findings in multisystem LCH. Mucosal lesions and otitis externa were almost exclusively seen in patients with multisystem disease, a finding that warrants further investigation. Both wild-type (WT) and mutated BRAF were found not only in multisystem LCH, but also in skin-only LCH. Two cases with rapidly resolving congenital lesions had WT BRAF. CONCLUSIONS:Late onset and a protracted course of skin lesions are associated with MS-LCH, whereas WT BRAF is found in rapidly resolving skin lesions.

journal_name

Pediatr Blood Cancer

journal_title

Pediatric blood & cancer

authors

Morren MA,Vanden Broecke K,Vangeebergen L,Sillevis-Smitt JH,Van Den Berghe P,Hauben E,Jacobs S,Van Gool SW

doi

10.1002/pbc.25834

subject

Has Abstract

pub_date

2016-03-01 00:00:00

pages

486-92

issue

3

eissn

1545-5009

issn

1545-5017

journal_volume

63

pub_type

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