Necrobiotic Xanthogranuloma

  • Adult disease
  • Skin has indurated yellowish plaques/nodules, starting on trunk but classically periorbital, scarring, ulceration
  • Ocular involvement common, inflammatory
  • Monoclonal IgG paraproteinemia (usually kappa) in 80%, BM suppression and myeloma possible
  • Pathology
    • Deeper palisaded granulomas (CD68+), Touton and bizarre foreign body giant cells, cholesterol clefts
  • Treatment
    • Based on paraprotein/malignancy
    • Cutaneous lesions recalcitrant to surgery