Juvenile xanthogranuloma

Juvenile xanthogranuloma is the most common type of non-Langerhans cell histiocytosis (see this term) characterized by the occurrence of one or more reddish or yellowish self-limiting and benign papules or nodules of several millimeters in diameter, usually appearing on the head and neck (but sometimes on the extremities and trunk) during the first year of life (or rarely in adulthood) and usually regressing spontaneously. Extracutaneous involvement has also been reported, involving most commonly the eye (uveal tract) but with other locations including the central nervous system, lung, liver, bones and endocrine glands, and may be associated with considerable morbidity.

Proptosis

An eye that is protruding anterior to the plane of the face to a greater extent than is typical.

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PMID (PMCID)
25804289	FEMALE
	Juvenile xanthogranuloma--a rare cause of unilateral orbital swelling in an elderly patient.
	Patel KH, El-Yassir M, Erzurum SA. Orbit. 2015;34(2):106-8.
	To report the clinical and pathologic features of an elderly patient with a unilateral orbital swelling and proptosis caused by Juvenile Xanthogranuloma diagnosed and confirmed by orbital biopsy.
11054007	MALE	Adult
	Juvenile xanthogranuloma of the orbit in an adult.
	Mencia-Gutierrez E, Gutierrez-Diaz E, Madero-Garcia S. Ophthalmologica. 2000;214(6):437-40.
	We present a case of juvenile xanthogranuloma (JXG) with unilateral involvement of the orbit and eyelid and proptosis, histologically confirmed in a 32-year-old man with a 1-year history of a pansinusitis and dacryoadenitis with rhinitis.