- Letterer-Siwe disease
- Let·ter·er-Si·we disease 'let-ər-ər-'sē-və- n an acute disease of children characterized by fever, hemorrhages, and other evidences of a disturbance in the reticuloendothelial system and by severe bone lesions esp. of the skullLetterer Erich (1895-1982)German physician. Letterer first described Letterer-Siwe disease in 1924. The author of several textbooks on pathology, he described the disease from the viewpoint of a pathologist.Siwe Sture August (1897-1966)Swedish pediatrician. Siwe presented another, independent description of Letterer-Siwe disease in 1933, this time from the clinical point of view.
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E. Letterer (20th century) and S. A. Siwe (1897-1966), German physicians* * *
a Langerhans cell histiocytosis of early childhood, of autosomal recessive inheritance, characterized by cutaneous lesions resembling seborrheic dermatitis, hemorrhagic tendency, hepatosplenomegaly with lymph node enlargement, and progressive anemia. If untreated it is rapidly fatal. Called also L-S d. and acute disseminated Langerhans cell histiocytosis.
Medical dictionary. 2011.