Fatal systemic juvenile xanthogranuloma with multiple central nervous system lesions

Abstract

Juvenile xanthogranuloma (JXG) is a rare and benign proliferative disease of histiocytes (1). Its systemic form is rarer and while the condition is benign, prognosis is poor (1). Extra-cutaneous involvement of JXG can be seen in the eyes, lungs, liver, spleen, adrenals, gonads, kidneys, bowels, retroperitoneum, and rarely, central nervous system (CNS) (1,2). In current literature, systemic JXG cases with multiple CNS lesions with fatal outcome are very rare. The purpose of this case study is to present magnetic resonance imaging (MRI) findings of an 18-month-old patient with JXG who presented with multiple CNS lesions and later developed skin lesions. To the best of our knowledge, there are no reports in the literature of cases where skin lesions developed after presentation of isolated CNS lesions.