Subdural effusion in a CNS involvement of systemic juvenile xanthogranuloma: A case report treated with vinblastin

Juvenile xanthogranuloma (JXG) is one of the most common non-Langerhans cell histiocytosis in children. Usually cutaneous, there are disseminated forms. However, neurological localization remains exceptional. A 7-month-old boy had been admitted for subdural effusion due to non-accidental head injury and skin nodular lesions. A biopsy of a skin lesion was considered suggestive of JXG. Skin, eyes, brain, lungs, liver, and testicles were involved. Systemic treatment of JXG was begun with vinblastine. It allowed the regression of skin, lung, and CNS lesions. At age of 11 years, he had not reappearance of the xanthogranuloma. This report emphasizes the possible presentation of xanthogranuloma with subdural effusions, the organs which should be examined in case of disseminated forms and the efficiency of vinblastin.