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Juvenile Xanthogranuloma

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Juvenile Xanthogranuloma

  • Epidemiology
  1. Onset in infants
  • Pathophysiology
  1. Benign histiocytic tumor of childhood (non-Langerhans Cell)
  • Signs
  1. Asymptomatic lesions
  2. Characteristics
    1. Yellow Papules and Nodules (some Nodules may be deep within the soft tissue)
    2. Typically appear as single lesions, but multiple lesions may occur
  3. Distribution
    1. Most common on the head and neck
    2. Also involve the upper torso and extremities
  4. Dermoscopy: "Setting Sun"
    1. Yellow-Orange background
    2. Foreground with yellow globules, white streaks and irregular vasculature
  • Differential Diagnosis
  1. See Langerhans Cell Histiocytosis
  • Labs
  • Histopathology
  1. Lipid-laden, foamy histiocytes
  2. Scattered Eosinophils and Lymphocytes
  3. Touton-type giant cells
    1. Multinucleated cells with fused epitheloid Macrophages
  • Course
  1. Lesions have onset in infancy and clear by adolescence
  • Management
  1. Solitary, Single Asymptomatic Xanthogranuloma
    1. Perform full body skin exam (evaluate for other associated lesions, findings)
    2. Observe for resolution
  2. Multiple Xanthogranulomas
    1. Abdominal Ultrasound
      1. Evaluate for hepatic xanthogranulomas (may present with Hepatomegaly, Jaundice)
    2. Ophthalmology Exam
      1. Evaluate for Eye involvement
  • References
  1. So (2020) Pediatr Dermatol 37(4):637-44 +PMID: 32468628 [PubMed]