Juvenile xanthogranuloma

• benign histiocytic tumours
• solitary or multiple
• often present at birth or in early infancy
• clinical features:
  • firm, small, red-brown nodules
  • common sites - face, neck, scalp
• management:
  • referral for specialist review is recommended (2,3)
    • there is an association between juvenile xanthogranuloma, neurofibromatosis and juvenile chronic myelogenous leukaemia
      • Zvulunov et al (3) found evidence that a concomitant finding of and juvenile xanthogranuloma in children with neurofibromatosis represents a true association, rather than a coincidence. A finding of juvenile xanthogranuloma in an infant with neurofibromatosis should alert a physician to a possible development of juvenile chronic myelogenous leukaemia
  • in general, no treatment of the skin lesions is required - there tends to be a spontaneous regression of lesions
  • ophthalmology examination is recommended because disseminated lesions can be associated with ophthalmic involvement

Reference:

1. Dermatology in Practice (2001), 9 (4), 20.
2. Jang KA et al.Juvenile chronic myelogenous leukemia, neurofibromatosis 1, and xanthoma. J Dermatol. 1999 Jan;26(1):33-5.
3. Zvulunov A, Barak Y, Metzker A.Juvenile xanthogranuloma, neurofibromatosis, and juvenile chronic myelogenous leukemia. World statistical analysis Arch Dermatol. 1995 Aug;131(8):904-8.