Juvenile Xanthogranuloma (JXG) is a rare benign histiocytic disorder.  Most patients with JXG only have skin disease and its first manifestations are little orange, sometimes reddish-brown bumps that are called papules (little bumps on the skin).  It often presents around the eyes, forehead or the face.  These papules tend to disappear on their own, or self-heal, without any kind of treatment.

Frequency of occurrence in the United States is unknown, but it may be higher than reported, since lesions occur early in life, may be misdiagnosed, and spontaneously regress.

JXG may be present at birth, but most often arises in infancy. Children younger than 6 months are more likely to have multiple lesions. Adult onset is reported infrequently.

The majority of lesions are confined to the skin, but other sites of involvement can include:

1. Eye
   Vision problems
   
   
2. Subcutaneous Soft Tissue and Muscle
   Small lumps under the skin
   
   
3. Brain and Spinal Cord
   Lumps pressing on normal tissue, possibly causing seizures or other problems
   
   
4. Lungs, Liver, Spleen and other organs
   Lumps interfering with normal function

The disease is usually more serious when several sites are affected.  The systemic forms can result in a more serious condition and although rare, death may occur.

A diagnosis of JXG is usually made following a biopsy of a lesion.  In the case of eye involvement, ocular ultrasound may also be used to help confirm the location of the intraocular or orbital lesions.

Currently, there is no standard treatment for JXG.  The severity and location of the involvement dictates the course of treatment.  JXG involving just the skin usually follows a benign course without treatment.  Systemic JXG may or may not require treatment.  However, surgery to remove the effected area maybe be necessary in some cases.  When surgery is not possible, chemotherapy, radiation therapy, and/or other treatments may be considered.