Abt-Letterer-Siwe-Disease

Last Updated: 2019-08-26

Author(s): -

  • 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
  • Biopsy
  • Capillitium (especially seborrheic areas)
  • Stem
  • Inguines
  • 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%

Topical Therapy

  • Elocom cream

 

Radiation therapy

  • PUVA therapy

 

Systemic Therapy

  • Prednisolone
  • Methotrexate
  • Cytostatic drugs

[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.