Juvenile xanthogranuloma (JXG) is a rare self-limiting non-Langerhans cell histiocytosis that typically affects infants and young children. Pathogenesis of JXG is not well-established and it is thought to be a reactive lesion. 1 It commonly presents as a solitary nodule or papule in the skin of the head, neck and upper trunk. It can also present as soft tissue lesions in the brain, lung, bone, testis, gastrointestinal tract, heart and eye. 2 Oral cavity involvement is exceedingly rare and less than fifty cases have been published in the English literature.
Wong et al. (Sun,) studied this question.