Juvenile Xanthogranuloma

• Figure 2.14 A: Juvenile xanthogranuloma (Courtesy of Dr. Michelle B. Bain) B: Juvenile xanthogranuloma (Courtesy of Dr. Paul Getz) C: LCH (Reprint from Morgan MB, Smoller BR, Somach SC. Deadly Dermatologic Diseases. New York, NY: Springer; 2007)

  Non-Langerhans cell histiocytosis with Touton giant cells; onset typically within first year of life
• Two types: micronodular (small, multiple) or macronodular (larger size, few in number)
• Presents as single or multiple firm, pink-red papulonodules with yellow hue on head/neck > trunk/upper extremities
• Regression typically seen in children (not in adults)
• 0.5% with ocular involvement : glaucoma, hyphema (may rarely result in blindness)
• Association with NF1 and juvenile myelomonocytic leukemia (JMML)