Gingival Juvenile Xanthogranuloma

Abstract

Juvenile xanthogranuloma (JXG) is a rare non-Langerhans type histiocytosis, that arises in the 1 st and 2 nd decade 1) . JXG has been earlier referred to as nevoxanthoendothelioma, because this xanthoma complex displayed as circumscribed, colored, chronic skin lesions composed of oval endotheliallike cells with various ranges of vascularity 2,3) . It was later classified as a benign histiocytic disorder most likely of dermal dendritic cell origin, but the specific histiocytic lineage is still controversial [4][5][6] .JXG is a pediatric disease with 45~71.0% of the cases diagnosed before the age 1 1,7) . The majority of JXG are cutaneous lesions. It is most common in the head and neck region, closely followed by the extremities and trunk 1,7,8) . Over 80% of the cutaneous JXG cases are solitary with a nearly 1:1 sex ratio, male being slightly more prevalent 1,7) .Whereas in multiple cutaneous lesions, 9 in 10 are male 1) . Extracutaneous lesions, especially those without a concurrent cutaneous lesion are extremely rare and have been seen most commonly in the submucosa and bone of the head and neck. Systemic visceral involvement may accompany the cutaneous lesions and are seen in the central nervous system, eye, lung, liver, pancreas, spleen, kidney, intestines and others 1,7,9,10) . Lesions are well circumscribed, firm nodules