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Juvenile xanthogranuloma (JXG) is a benign, self-limiting disease, which is a subtype of class II histiocytosis that occurs most frequently in infants and children, but sometimes also affects adults. This condition usually presents with cutaneous lesions in the head and neck area, trunk, extremities, but is very rare in the external auditory canal. Occasionally, it can be found as extracutaneous lesions, including oral cavity, nasal cavity, trachea, glottis, subglottis and other visceral organs. In most cases, no treatment is required because of the self-regressive nature of skin lesions, but recurrence after excision has been reported in some cases of the adult type JXG or in oral cavity lesions. We report, with a review of the related literature, a case of JXG located in the right side of external auditory canal.
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