Systemic Juvenile Xanthogranuloma

JXG is a rare disease that belongs to a group of illnesses called Histiocytosis that in most cases attack children under ten year old. Histiocytic disorders are associated with an excess of white blood cells (histiocytes) that are part of the immune system and whose function is to fight infections. For unknown reasons, the histiocytes cluster together forming bumps (lesions or tumors) that attack different organs of the human body. There are three manifestations of this disease:

 

1) Cutaneous JXG, where lesions are only found in the skin and usually follows a benign course without treatment.

2) Systemic JXG, where lesions are present in multiple organs such as skin, eye, lung, spinal cord, brain, etc. and surgery, chemotherapy, radiation, or steroids are usually required. This expression of the condition is very serious and survival depends on the extend of non-skin disease.

3) Ocular JXG: where lesions are present in the eyes of the patient and they may or may not be accompanied by skin lesions.

 

According to the article entitled "Uncommon Histiocytic Disorders" by Weitzman and Jaffe of the Hospital for Sick Children from Toronto, Canada, the chances of Cutaneous JXG becoming Systemic are low, around 4% to 10%.

Due to the infrequency of the disease and its misdiagnoses is very difficult to obtain accurate numbers of the people affected by it. However, its is roughly estimated that Cutaneous JXG attacks 1 in a million children and Systemic JXG affects 1 in 10 million children.


The cause of Histiocytosis is not known and because this disease is so rare, no large studies have been performed, and there is no established, proven treatment or cure.