TY - CHAP M1 - Book, Section TI - Letterer-Siwe Disease (LSD) A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas PY - 2019 T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - It is a childhood immunologic disorder characterized by pathologic proliferation of histiocytes caused by the Langerhans cells. It is one of the four recognized clinical syndromes of Langerhans cell histiocytosis (LCH). It causes approximately 10% of LCH disease and is the most severe form. It is characterized by skin lesions (scaly skin involving the scalp, ear canals, and abdomen), lymphadenopathy, osteolytic lesions, and hepatosplenomegaly. Other organs involved are the spleen, brain, lungs, and bones. The presence of diabetes insipidus (DI) must be considered. Presents nonmalignant growths that represent accumulation of histiocytes. It is associated with a poor prognosis (70% mortality). SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accessanesthesiology.mhmedical.com/content.aspx?aid=1164076789 ER -