Fatal systemic juvenile xanthogranuloma with multiple central nervous system lesions

Dear Editor,Juvenile xanthogranuloma (JXG) is a rare and benignproliferative disease of histiocytes (1). Its systemic formis rarer and while the condition is benign, prognosis ispoor (1). Extra-cutaneous involvement of JXG can beseen in the eyes, lungs, liver, spleen, adrenals, gonads,kidneys, bowels, retroperitoneum, and rarely, centralnervous system (CNS) (1,2). In current literature, systemicJXG cases with multiple CNS lesions with fatal outcomeare very rare. The purpose of this case study is topresent magnetic resonance imaging (MRI) findings ofan 18-month-old patient with JXG who presented withmultiple CNS lesions and later developed skin lesions.To the best of our knowledge, there are no reports in theliterature of cases where skin lesions developed afterpresentation of isolated CNS lesions.