Abt Letterer Siwe disease
Last Updated: 2023-07-07
Author(s): Anzengruber F., Navarini A.
- Abt u. Letterer 1924
- Siwe 1933
- Histiocytosis X
- Acute reticuloendotheliosis
- Aleukaemic reticulosis
- Acute disseminated juvenile form
- Letterer-Siwe disease
- Acute, generalised, malignant progression of Langerhans cell histiocytosis, which usually occurs in infants or congenital children.
- Papulosquamous, eczema-like exanthema, partly with necrotic involvement and petechiae.
- Extracutaneous involvement: fever, anaemia, lymphadenopathy, hepatosplenomegaly, pulmonary involvement, osteolyses (skull, ribs, extremities, ilium).
- Clinical picture
- Capillitium (especially seborrhoeic areas)
- Lymphohistiocytic infiltrate with often atypical cells. Epidermotropism of eosinophilic cells.
- Under the electron microscope, Birbeck granules (Langerhans cell granules) can be seen.
Lethal if untreated. The 5-year survival rate with therapy is approx. 50%.
 Ben Slama L, Ruhin B, Zoghbani A. [Langerhans cell histiocytosis]. Rev Stomatol Chir Maxillofac. 2009;110; 287-289.
 Nezelof C, Basset F. Langerhans cell histiocytosis research. Past, present, and future. Hematol Oncol Clin North Am. 1998;12; 385-406.