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A CASE OF ANGIOKERATOMA CORPORIS DIFFUSUM (FABRY): CLINICAL AND ELECTRON MICROSCOPIC STUDY Sadanori NAGAO 1 , Kyuei SUGAWARA 1 , Yoshio SATO 2 , Morio MASAKI 3 , Yoshio YAGINUMA 3 1Department of Dermatology, Fukushima Medical College 2Department of the First Internal Medicine, Fukushima Medical College 3Senior Student of Fukushima Medical College pp.531,567-575
Published Date 1971/6/1
DOI https://doi.org/10.11477/mf.1412200814
  • Abstract
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 A 18-year-old man with angiokeratoma corporis diffusum (Fabry) was studied clinically and light and electronm icroscopically. The patient showed typical exanthemas distributed mostly between the navel and the knees, erythromelalgialike symptom, hypohidrosis, proteinuria, impaired concentration power of the kidneys, ankle clonus and fever.

 Light microscopy of the biopsied skin from right trochanter area revealed many osmiophilic granules within nedothelial cells and connective tissue cells after the specimen was fixed with OsO4 and embedded in epon, but not after using formalin and paraffin.

 Electron microscopic study revealed that the osmiophilic granules were found within endothelial cells, pericytes, connective tissue cells and those of perineurium. Four types of ultrastructure were seen in th egranules; lamellar structure with 54-65A periodicity, round arrangements, finely granular structure with moderately high electron density andamorphous structure with high electron density. Frequently peculiar bundles were seen within endothelial cells. The bundles sometimes contained the four types of ultrastructure mentioned above and were connected peripherally with fine cytoplasmic filaments which were dispersed irregularly within the cells.

 These findings suggest that there are close relationship among the fine cytoplasmic filaments the peculiar bundles described by us and the characteristic lamellar structure, concerning the development of the granules in Fabry's disease.


Copyright © 1971, 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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