Langerhans Cell Histiocytosis Preceding the Development of Juvenile Xanthogranuloma: A Case and Review of Recent Developments

Abstract

Langerhans cell histiocytosis (LCH) and juvenile xanthogranuloma (JXG) both belong to the histiocytosis group of disorders, which have varied prognostic and clinical significance. Their normal cellular counterparts share a common CD34-positive hematopoietic stem cell precursor that matures along CD14-negative or -positive pathways. Rare cases of LCH and JXG show overlapping findings, suggesting that this divergent maturation is not irreversible. We report a case of an infant diagnosed with cutaneous LCH shortly after birth. Two years after diagnosis, a recurrent lesion in his external auditory canal contained lipidized cells with CD68 and factor XIIIa expression and lacked Birbeck granules and CD1a, consistent with JXG. Our case and previous reports of this phenomenon raise the question of a histogenic relationship between LCH and JXG, and we speculate that the lesional cells of origin are subjected to therapy-induced modulation that results in a varied differentiation.