Juvenile Xanthogranuloma of Middle Ear and External Auditory Canal: A Rare Case Report

Juvenile xanthogranuloma (JXG) is a benign self-limiting non langerhans cell histiocytic lesion of unknown etiology and pathogenesis. It commonly affects infants and adolescents only 15% cases reported from the adult population. It characteristically appears as yellow to brown solitary papule, affecting the skin of head, neck and trunk region. Extra cutaneous JXG are reported from various sites including central nervous system, gastrointestinal system, kidney, larynx, tongue with single case report from tympanic membrane and external auditory canal. We describe a very rare case of adult JXG occurring as multiple papules from the external auditory canal and granulations from middle ear, which presented as persistent otorrhea. We discuss the clinical presentation, imaging, pathological findings and review of literature for this case. To the best of our knowledge this is first reported case of JXG from the middle ear cavity.