Letterer-Siwe disease


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Letterer-Siwe disease

[′led·ə·rər ′zē·və di‚zēz]
(medicine)
A fatal disease of infants and young children, of unknown etiology, characterized by hyperplasia of the reticuloendothelial system without lipid storage.
McGraw-Hill Dictionary of Scientific & Technical Terms, 6E, Copyright © 2003 by The McGraw-Hill Companies, Inc.
References in periodicals archive ?
Acute disseminated LCH (Letterer-Siwe disease) usually occurs in infants, but can be seen in older children.
Pauline, 32, had a rare condition called Letterer-Siwe disease which stunted her growth so she is only 4ft 10in.
The differential was given as osteomyelitis, which was considered unlikely because of the sharp margins, hemangioma, and histiocytosis, or Letterer-Siwe disease. A bone scan, which was obtained to assess the possibility of disease in multiple bones, showed increased uptake in the right mandible only (Figure, left and right).
This disease is a spectrum of three sub-types including eosinophilic granuloma, hand-schuller-christian disease and letterer-siwe disease. We are presenting a case of 6 year old twin brothers who came with complaints of multiple painless swellings over the scalp.