Lingual juvenile xanthogranuloma in a woman: a case report

We present a rare case of oral juvenile xanthogranuloma in a 49-year-old Caucasian woman. The histopathologic diagnosis of the lingual neoformation was histiocitary proliferation with the presence of giant cells, Touton type, compatible with juvenile xanthogranuloma.To establish an accurate diagnosis, microscopic evaluation and immunohistochemical staining are necessary. Dentists, dermatologists and general practitioners may be the first to recognize this rare condition during the inspection of the oral cavity.Juvenile xanthogranuloma is an uncommon non-Langerhans cell histiocytosis that usually occurs during infancy and early childhood. This lesion was first reported by Adamson in 1905 [1], who used the term congenital xanthoma multiplex. This pathological condition has subsequently been reported under various names such as nevoxanthoendothelioma, juvenile xanthoma or xanthoma tuberosum [2]. Its modern name was introduced by Senear [3] in 1936 and popularized by Helwig and Hackney in 1952 [4].The pathogenesis is unknown, although the disease is believed to be a reactive rather than a neoplastic process. It is caused by the proliferation of plasmacytoid monocytes in response to an unknown etiologic agent, possibly either physical or infectious [5].Juvenile xanthogranuloma is a benign and self-healing disorder which is characterized by the presence of single or multiple raised cutaneous nodules, yellow-brown to reddish in color and usually measuring from a few millimeters to a few centimeters in diameter. Sometimes superficial telangiectasias and an erythematous border may also occur [6]. The most involved cutaneous regions are the neck, head and upper trunk, followed by the extremities.Juvenile xanthogranuloma is not limited to cutaneous sites, however. The eye is the most common extracutaneous site, with other affected organs including the lung, kidney, pericardium, colon, central nervous system, liver, spleen, eye/orbit, bone, kidneys, adrenal glands, testis and o