Giant congenital juvenile xanthogranuloma

Congenital giant juvenile xanthogranuloma is an uncommon lesion that apparently has benign biologic behavior in most reported patients. In one of our three patients it was necessary to perform histochemical reactions with CD 1 (-), MAC 387, and electron microscopy to rule out Langerhans cell histiocytosis. The other patients had a typical histologic picture, with foamy histiocytes and Touton cells in association with lymphocytes and eosinophils. Comments about treatment are also made.