Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis and is fairly common, often affecting infants and young children. Cutaneous lesions usually resolve spontaneously and most patients have an unremarkable course. Ocular JXG can occur and has an increased incidence in those 2 years of age or younger or in those with multiple lesions. Ocular involvement can be associated with many potential complications including blindness. There is an association of JXG with juvenile myelomonocytic leukemia and neurofibromatosis type 1 (NF1).
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