Letterer-Siwe disease
ICD-10 Code
C96.0
Letterer-Siwe disease
Definition
A multifocal, multisystem form of Langerhans-cell histiocytosis. There is involvement of multiple organ systems including the bones, skin, liver, spleen, and lymph nodes. Patients are usually infants presenting with fever, hepatosplenomegaly, lymphadenopathy, bone and skin lesions, and pancytopenia.
Also known as acute and disseminated Langerhans cell histiocytosis, acute disseminated Langerhans cell histiocytosis, letterer-Siwe disease, multifocal multisystem Langerhans cell histiocytosis — per MONDO