Congenital Letterer-Siwe disease

Abstract

Langerhans' cells histiocytosis represent a group of rare disorders characterized by reactive infiltrates that usually are composed of varying proportions of histiocytes, lymphocytes, plasma cells, eosinophils, and neutrophils. Letterer-Siwe disease is the most severe form of histiocytoses. It commonly presents within the first year of life and, occasionally, in neonates. We present clinical and histopathological pictures in a neonate with unrecognized congenital Letterer-Siwe disease. The child died due to diffuse infiltration of lungs with histiocytic cells.