jxg disease - HEALTHIES
JXG is considered a rare disease in itself, but is the most common type of non-Langerhans histiocytosis. The incidence of juvenile xanthogranuloma is estimated to be 1 per million in children, however it is probably underdiagnosed. Up to 10% of patients with neurofibromatosis type I may develop JXG.
Understanding the Context
A juvenile xanthogranuloma, or JXG, is a benign skin lump or bump caused by a collection of cells called histiocytes. These may be red, orange or tan at first, but over time may become more yellow in color. Juvenile xanthogranuloma (JXG) is a rare type of histiocytic disorder that mostly occurs in children (average age 2 years), but can rarely occur in adults as well, in which case it is called adult xanthogranuloma (AXG). JXG presents in the first two years of life as a solitary, reddish or yellowish skin papule or nodule, most often on the head, neck, or upper trunk.
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Key Insights
Extracutaneous or systemic forms are exceedingly rare and can be associated with considerable morbidity. An overview of JXG will be presented here. Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis and is fairly common, often affecting infants and young children. Cutaneous lesions usually resolve spontaneously and most patients have an unremarkable course. JXG is a benign idiopathic cutaneous granulomatous tumor and the most common form of non-Langerhans cell histiocytosis (non-LHC).
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[3] The lesions appear as orange-red macules or papules and are usually located on the face, neck, and upper trunk. A juvenile xanthogranuloma (JXG) is a benign (non-cancerous) skin bump. JXGs can appear anywhere on the skin and at any age but most often develop within a child’s first years of life. Juvenile xanthogranuloma (JXG) is a rare benign histiocytic proliferation that develops in infants and young children. JXG is the most common form of non-Langerhans’ cell histiocytoses. It is characterized by the presence of Touton giant cells.