[Congenital manifestations of juvenile xanthogranuloma (large nodular form)]

An infant girl presented at birth with multiple, large nodular xanthogranulomas. Her monozygotic twin sister was not affected. The congenital tumors were up to 1.5 cm in diameter, done-shaped and mainly located on the head and the upper half of the body. Histologically the cells were characterized as CD68+ non-Langerhans histiocytes. Follow-up for 18 months showed no new tumors and regression of the existing ones. No extra-cutaneous manifestations were observed. Knowledge of the differential diagnosis, especially the group of Langerhans cell histiocytosis, is essential for prognosis estimation.