A Case of Juvenile Xanthogranuloma Developed in a Neurofibromatosis Child with Family History

Juvenile xanthogranuloma (JXG) is a fibrohistiocytic proliferative disorder predominant in infancy and childhood. It is a benign and transient skin lesion that usually appears in the cephalic area. Recently, the concurrent finding of neurofibromatosis (NF), juvenile chronic myelogenous leukemia (JCML), and JXG has been repeatedly reported. Especially, the family history of NF may represent a risk factor for the development of JCML in patients with NF and in patients with NF and JXG. So, a finding of JXG and NF in infants should alert the physician to a possible development of JCML. We report a case of JXG developed in a neurofibromatosis child with family history. (Korean J Dermatol 2008;46(4):547∼549)