Intraoral and Cutaneous Juvenile Xanthogranuloma

Abstract

A three-year-old male with histologically proven intraoral and cutaneous juvenile xanthogranuloma is described. In our review of the literature published in English we were unable to find a similar case report. The clinical and histologic features of this disorder are discussed.

Xanthogranuloma: Report on Clinical and Histologic Findings in 64 Patients

“…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.…”

Juvenile xanthogranuloma occurring in the oral cavity: case report and histopathological findings

“…Many lipid droplets without limiting membrane in the oral cavity have been of the uni-(arrows) are observed in the cytoplasm, but no Langerhans" cell granules were observed, foca! type (6), Of the 11 previously re-(Original magnification: x 10,000) ported cases of JXG with onset in the oral cavity only the one case of OSSOFF et al (9) was muUifocal, and in this case lesions were noted in both the oral cavity and dermis. In such a case the diagnosis is easily made, but since most cases of JXG in the oral cavity are unifocal, all are resected, combining diagnosis and therapy JXG must be differentiated from other conditions, in particular histiocytosis X, because both the treatment approach and prognosis differ.…”

Juvenile xanthogranuloma: Case report with immunohistochemical identification of early and late cytomegalovirus antigens.

“…Although one report has suggested that it may represent a reactive virally-induced lesion associated with cytomegalovirus (CMV) infection (8), this has not been confirmed by others (5). JXG in the oral cavity is rare, and only 15 cases have been reported in the English-language literature (7,(9)(10)(11)(12)(13)(14)(15)(16). The present paper reports one additional case of JXG and discusses its possible association with CMV infection.…”

“…Oral JXG is rare and only ten cases have been reported in the English-language literature (7,(9)(10)(11)(12)(13)(14)(15)(16). Clinically, oral JXG may be misdiagnosed as pyogenic granuloma, adenocarcinoma, fibro-epithelial polyp, lipoma, granular cell tumor, lymphoid aggregate, or verruciform xanthoma (9,11,14).…”