Juvenile Xanthogranuloma Causing Bilateral Eyelid Swelling and Severe Ptosis in a Nine- Year Old Male

Abstract

Background: Juvenile Xanthogranulomas (JXG)are rare benign lesions that belong to the large group of non-Langerhan cell histiocytoses.¹The most common ocular presentation is on the Iris.² They occur predominantly in children (40%-75%) in the first year of life ² with >15-20% having lesions at birth.³Eyelid lesions are extremely rare and tend to present later in life.³ There are two existing variants: a) multiple 2 – 5 mm dome shaped papules and b) one or more large nodules 1 - 2 cm in size. There is a slight male preponderance with a male to female ratio of ¹.7:1 and prevalence of ¹ in 1 million. ².