Letterer–Siwe disease
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.
Presentation[edit | edit source]
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]
Cause[edit | edit source]
Oncogenic mutation of BRAF 50-70% cases[citation needed]
Diagnosis[edit | edit source]
![[icon]](/wiki/File:Wiki_letter_w_cropped.svg){kind=small} | This section is empty. You can help by adding to it. (July 2017) |
Prognosis[edit | edit source]
The disease is often rapidly fatal, with a five year survival rate of 50%. The development of thrombocytopenia is a poor prognostic sign.[1]
References[edit | edit source]
- ↑ 1.0 1.1
- ↑ RESERVED, INSERM US14 -- ALL RIGHTS. "Orphanet: Letterer Siwe disease". www.orpha.net. Retrieved 2017-05-19.
{{cite web}}: CS1 maint: numeric names: authors list (link) - ↑ "Langerhans cell histiocytosis | DermNet New Zealand". www.dermnetnz.org. Retrieved 2017-05-19.
External links[edit | edit source]
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