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AN AUTOPSY CASE OF LETTERER-SIWE'S DISEASE Shoichi KINEBUCHI 1 , Akira MATSUNAGA 2 , Tsunehiko WATANABE 3 1Depaetment of Dermatology, Teishin Hospital 2Department of Pediatrics, Tokyo Teishin Hospital. 3Department of Pathology, Tokyo Teishin Hospital. pp.881-894
Published Date 1967/8/1
DOI https://doi.org/10.11477/mf.1412200199
  • Abstract
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A 6-month-old boy of typical Letterer-Siwe's disease with various skin manifestions com-posed of papules of red and yellowish-red colors, crusts, erosions and bleeding spots was reported. The eruption started with fever and accompanied with bilateral cervical lymphad-enopathy, anemia and hepatosplenomegaly. Histologic section of the skin eruption showed a dense infiltrate of reticulum cells and pronounced bleeding in the upper dermis and atrophy of the epidermis.

Slight leukocytosis, anemia red blood cell count, 2,810,000 and thrombocytopenia were proved, but bleeding time and blood coagulation time were within normal lmits. Roentgeno-grams of the bones did not show any abnormalities.

In spite of treatmens with adrenocortical steroids, antibiotics and blood transfusion, the disease took an acute course to its termination after temporary remission of the symptoms.

Histologic specimens of the thymus, lymph nodeb, liver, spleen and intestines obtained by autopsy showed the pathologic picture in their lymph follicle which was composed of proliferation of reticulum cels with increased reticulum fibers and slight deposition of Su-dan III-positive substances in some parts, and proliferation of histiocytes with scanty reticulum fibers and dense deposition of Sudan III-positive substances in other parts.

A review on this disorder, Hand-Schuller-Christian's disease and eosinophilic granuloma of the bone was performed.


Copyright © 1967, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1324 印刷版ISSN 0021-4973 医学書院

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