Last Updated: 2019-08-26
- Abt u. Letterer 1924
- Siwe 1933
- Histiocytosis X
- Acute reticuloendotheliosis
- Aleukemic reticulosis
- Acute disseminated juvenile form
- Letterer-Siwe disease
- Acute, generalized, malignant form of Langerhans cell histiocytosis, which usually occurs in infants or young children.
- Papulosquamous eczema-like exanthema, partly with necrotic infestation and petechia.
- Extracutaneous infections: fever, anemia, lymphadenopathy, hepatosplenomegaly, lung infestation, osteolysis (skull, ribs, extremities, iliac bone).
- Clinical image
- Capillitium (especially seborrheic areas)
- Lymphohistiocytic infiltrate with often atypical cells. Epidermotropism of eosinophilic cells.
- Birbeck granules (Langerhans cell granules) can be seen under the electron microscope.
Untreated lethal. The 5-year survival rate under therapy is approx. 50%
- Elocom cream
- PUVA therapy
- Cytostatic drugs
 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.