Bilateral juvenile xanthogranuloma of the buttocks - An unusual location: Case report

Juvenile xanthogranuloma (JXG) predominantly occurs in infancy and childhood. Juvenile xanthogranulomas (JXGs) are asymptomatic; benign; self-healing; self-limiting red, yellow, or brown papules and nodules composed of histiocytic cells. Most common site of occurrence is skin of the head and neck region and trunk but occurrence at rare sites such as nasal cavity, limbus of the eye, iris of the eye, anterior abdominal wall and tibia had been reported in literature. JXG is the most common form of non?Langerhans cell histiocytosis. Approximately 35% of cases of juvenile xanthogranuloma (JXG) occur at birth, with as many as 71% of cases occurring in the first year. The mean age at presentation is 22 months. Most JXGs resolve by age 5 years. Despite the term juvenile in the disease name, 10% of cases manifest in adulthood. Most of the lesions are solitary. Juvenile xanthogranuloma is a histiocytic inflammatory disorder capable of presenting as different histological patterns. The classic form consists of sheets of foamy histiocytes and numerous multinucleated Touton-type giant cells containing no to very few mitotic figures. We present a case of large juvenile xanthogranuloma (JXG) in a 7 year old child involving an unusual location and bilateral involvement of both the buttocks.