Juvenile xanthogranuloma and peripheral neurofibromatosis.

Abstract

An 8-month-old girl was first observed for the presence of a dozen of café-au-lait spots (Fig. 1). The history and physical examination of family members were negative. Physical examination showed 10 round, brown uniform, 1-4 cm in diameter, patches randomly distributed throughout the skin surface. Lisch nodules were absent in her parents. These findings led to diagnose peripheral neurofibromatosis probably due to fresh mutation. At the age of 13 months three yellow plaques of 4-10 mm were observed in the left parietal region (Fig. 1, 2). The lesions had appeared 2 months before and initially had a reddish color, toned to yellow within a month. An ophthalmology consultation showed no alterations. The diagnosis was juvenile xanthogranuloma in a patient with peripheral neurofibromatosis. Her parents were informed of their probable spontaneous regression within two years.