Abt Letterer Siwe disease

• 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%.

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.