Fatal systemic juvenile xanthogranuloma with multiple central nervous system lesions

Abstract

Dear Editor,
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.