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

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

doi:10.1186/s12887-019-1490-y

Michaela Höck, Gabriele Kropshofer + Show 5 more

Open Access

https://doi.org/10.1186/s12887-019-1490-y

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Abstract

BackgroundJuvenile 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 presentationTwo 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.ConclusionJXG 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.

Highlights

Juvenile xanthogranuloma (JXG) belongs to the heterogeneous group of non-Langerhans cell histiocytosis and is caused by an accumulation and proliferation of macrophages
Cutaneous JXG is generally regarded as a self-limited condition, systemic JXG may be associated with significant morbidity and occasional deaths so that aggressive medical care is necessary [20]
Juvenile xanthogranuloma belongs to the heterogeneous group of non-Langerhans cell histiocytoses and generally tends to have a good prognosis

Summary

Introduction

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. 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. Juvenile xanthogranuloma (JXG) is a rare ‘histiocytic’ disorder and belongs to the broad group of non-Langerhans cell histiocytosis [1]. In the Kiel Paediatric Tumor Registry spanning 35 years JXG accounted for 129 (0.5%) out of 24.600 paediatric lesions.

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