Letterer–Siwe disease

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Letterer–Siwe disease
Synonyms Acute and disseminated Langerhans cell histiocytosis
Autosomal recessive - en.svg
This condition is inherited in an autosomal recessive mannerre
Classification and external resources
Specialty oncology
ICD-10 C96.0
ICD-9-CM 202.5
ICD-O 9722/3
OMIM 246400
DiseasesDB 5906
MeSH C538636
Orphanet 99870

Letterer–Siwe disease 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.[1] Prevalence is estimated at 1:500,000 and the disease almost exclusively occurs in children less than three years old.[2] The name is derived from the names of Erich Letterer and Sture Siwe.

Clinical Presentation[edit]

.Letterer-Siwe is characterized by skin lesions, ear drainage, lymphadenopathy, osteolytic lesions, and hepatosplenomegaly. The skin lesions are scaly and may involve the scalp, ear canals, and abdomen.[3]


oncogenic mutation of BRAF 50-70% cases



The disease is often rapidly fatal, with a five year survival rate of 50%. The development of thrombocytopenia is a poor prognostic sign.[1]


  1. ^ a b "Langerhans Cell Histiocytosis - Hematology and Oncology - Merck Manuals Professional Edition". Merck Manuals Professional Edition. Retrieved 2017-05-19. 
  2. ^ RESERVED, INSERM US14 -- ALL RIGHTS. "Orphanet: Letterer Siwe disease". www.orpha.net. Retrieved 2017-05-19. 
  3. ^ "Langerhans cell histiocytosis | DermNet New Zealand". www.dermnetnz.org. Retrieved 2017-05-19.