“…Lamb and Lain reported the first case of xanthogranuloma with visceral involvement in 1937 (9), This 3-month-old had multiple cutaneous lesions, dyspnea, and fever, and many pulmonary densities on chest radiograph. Subsequently, biopsy-proved lesions have been demonstrated in numerous extracutaneous sites, including the eye, lung, testicle, bone, periosteum, pericardium, myocardium, oropharynx, salivary gland, vulva, skeletal muscle, central nervous system, liver, spleen, and omentum (1,2,(12)(13)(14)(15)(16)(17)(18). Xanthogranulomas have also been associated with a number of systemic disorders, such as urticaria pigmentosa, diabetes mellitus, neurofibromatosis, cytomegalovirus infection, and leukemia (1^22), Although the pathogenesis of xanthogranulomas is unclear, pathologists have suggested a relationship to the histiocytosis X syndromes.…”