Abt Letterer Siwe disease

Last Updated: 2023-07-07

Author(s): Anzengruber F., Navarini A.

ICD11: 2B31.2Y

  • 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
  • Biopsy

  • Capillitium (especially seborrhoeic areas)
  • Trunk
  • Leaves

  • 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%.

[1]          Ben Slama L, Ruhin B, Zoghbani A. [Langerhans cell histiocytosis]. Rev Stomatol Chir Maxillofac. 2009;110; 287-289.

[2]          Nezelof C, Basset F. Langerhans cell histiocytosis research. Past, present, and future. Hematol Oncol Clin North Am. 1998;12; 385-406.