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BMC Pediatrics
Published in: BMC Pediatrics 1/2019

Open Access 01-12-2019 | Langerhans Cell Histiocytosis | Case report

The various clinical spectra of juvenile xanthogranuloma: imaging for two case reports and review of the literature

Authors: Michaela Höck, Bernhard Zelger, Gisela Schweigmann, Barbara Brunner, Bettina Zelger, Gabriele Kropshofer, Ursula Kiechl-Kohlendorfer

Published in: BMC Pediatrics | Issue 1/2019

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Abstract

Background

Juvenile xanthogranuloma (JXG) belongs to the heterogeneous group of non-Langerhans cell histiocytosis and is caused by an accumulation and proliferation of macrophages. In the majority of cases JXG is a disorder of early childhood presenting during the first 2 years of life. The typical presentation is a solitary reddish or yellowish skin papule or nodule with spontaneous regression and no need for treatment.

Case presentation

Two infants with an atypical presentation of JXG, one with multiple blueberry muffin rash-like skin lesions and the other with severe multi-systemic involvement, are reported. Diagnosis was established by skin biopsy including histological work-up and immunostaining, where markers for macrophages (CD68 and CD163) exhibited significant reactivity.

Conclusion

JXG is the most common of the non-Langerhans cell histiocytosis. The typical presentation is a solitary skin lesion. The purpose of this report is to familiarize paediatricians with an unusual variant of this entity in order to facilitate early diagnosis and raise awareness for possible visceral complications and associated medical conditions.
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Metadata
Title
The various clinical spectra of juvenile xanthogranuloma: imaging for two case reports and review of the literature
Authors
Michaela Höck
Bernhard Zelger
Gisela Schweigmann
Barbara Brunner
Bettina Zelger
Gabriele Kropshofer
Ursula Kiechl-Kohlendorfer
Publication date
01-12-2019
Publisher
BioMed Central
Published in
BMC Pediatrics / Issue 1/2019
Electronic ISSN: 1471-2431
DOI
https://doi.org/10.1186/s12887-019-1490-y

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