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FIGURE 88.9 Urticaria pigmentosa with Darier sign Mastocytoma, Urticaria Pigmentosa These are red/brown papules that urticate For additional information please see Chapter 69 Rash: Neonatal Juvenile Xanthogranulomas Juvenile xanthogranuloma JXGs can be confused with urticaria pigmentosa or xanthomas Numerous yellow or reddish-brown papules appear on the face and upper trunk in the first year of life The number of lesions may increase until the child is 18 months to years of age Serum lipid levels are normal, and the Darier sign (urtication after scratching) is negative The lesions often disappear spontaneously after years of age; therefore, intervention is generally unnecessary When JXGs are multiple, particularly on the head and neck, evaluation of the eyes for intraocular JXGs is recommended because of their potential for visual impairment The presence of JXGs in a young child with neurofibromatosis (type 1) has been a marker associated with an increased risk of juvenile myelomonocytic leukemia