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Xanthoma disseminatum. An unusual histiocytosis syndrome.

作者信息

Giller R H, Folberg R, Keech R V, Piette W W, Sato Y

机构信息

Department of Pediatrics, University of Iowa College of Medicine, Iowa City.

出版信息

Am J Pediatr Hematol Oncol. 1988 Fall;10(3):252-7.

PMID:3140686
Abstract

The histiocytoses are a diverse group of illnesses that present a variety of diagnostic and therapeutic dilemmas. In this article, we describe the case of an 8-year-old boy with xanthoma disseminatum, a histiocytic disorder involving the skin, eyes, and brain. In our discussion, we have emphasized the clinical, pathologic, and radiographic features that distinguish this entity from clinically similar juvenile xanthogranuloma and Langerhans' cell histiocytoses (histiocytosis X) such as the Hand-Schuller-Christian syndrome. The importance of differentiating the various histiocytoses, in view of their varying natural histories and therapeutic responsiveness, is reviewed in relation to the new classification system for these disorders proposed by the Histiocyte Society.

摘要

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