Congenital large nodular juvenile xanthogranulomas

An infant girl presented at birth with multiple, large nodular xanthogranul omas. Her monozygotic twin sister was not affected. The congenital tumors w ere up to 1.5 cm in diameter, done-shaped and mainly located on the head an d the upper half of the body. Histologically the cells were characterized a s CD68+ non-langerhans histiocytes. Followup for 18 months showed no new tu mors and regression of the existing ones. No extracutaneous manifestations were observed. Knowledge of the differential diagnosis, especially the grou p of Langerhans cell histiocytosis, is essential for prognosis estimation.