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Letterer-Siwe disease

Disease definition

Letterer-Siwe syndrome (LSS) is the acute disseminated multisystem form of Langerhans cell histiocytosis (see this term) characterised by proliferation of nonlipid histiocytes in the viscera and bones.


Classification level: Subtype of disorder
  • Synonym(s):
    • Acute and disseminated Langerhans cell histiocytosis
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: -
  • Age of onset: Infancy, Neonatal
  • ICD-10: C96.0
  • OMIM: 246400
  • UMLS: C0023381
  • MeSH: C538636
  • GARD: -
  • MedDRA: 10024265

Detailed information

Article for general public

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