Inhibition of cellular apoptosis appears to play a minor role in the growth of xanthogranulomas. The appearance of giant cells and foamy, lipid-laden histiocytes occurs late, and apparently are secondary events, possibly in response to cytokine production by histiocytes. Serum lipids are normal and remain normal. JXG occurs in Caucasians about 10 times more frequently than in blacks. Patients usually present in infancy or early childhood with an asymptomatic, smooth, round, yellow papule or papules.
Lesions usually are asymptomatic.
Mucous membrane, tongue, and palm and sole involvement are rare. A papular and nodular form of JXG has been described. Juvenile chronic myelogenous leukemia has been observed in association with multiple JXGs, and the incidence is especially high in patients with coexistent neurofibromatosis. A skin biopsy may be performed, both for diagnosis and cosmesis. This will usually consist of complete excision of the papule or nodule.