ISSN:
1432-069X
Keywords:
Paraproteinemia (IgG Kappa)
;
Protein crystalloids
;
Xanthomatosis
;
Corneal crystalline deposits
;
Light-microscopy
;
Electronmicroscopy
;
Paraproteinämie (IgG Kappa)
;
Proteinkristalle
;
Xanthomatosis
;
korneale Kristallablagerungen
;
Lichtmikroskopie
;
Elektronenmikroskopie
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Description / Table of Contents:
Zusammenfassung Es wird über einen 57jährigen Patienten mit Paraproteinämie (IgG Kappa), mit ödematöser Induration und Xanthomatisation der Haut, meist im Bereich des Rumpfes, sowie mit kristallinen Einlagerungen in der Hornhaut berichtet. Lichtmikroskopisch findet man neben einem Elastika-Schwund und einer Verminderung der Kollagenfasern mono- bzw. multinukleäre Zellen mit einem zart gestreiften Cytoplasma, das außerdem sudanophiles teils doppelbrechendes Material enthält. Elektronenmikroskopisch entsprechen diese Zellen vornehmlich Histiocyten mit zahlreichen, kristallinen, intracytoplasmatisch gelagerten Gebilden, die mit Pronase P verdaubar sind. Identische Gebilde, wenn auch seltener, kommen in den Keratinocyten vor. Die Verteilung der sauren Phosphatase-Aktivitäten in den zerfallenden korialen Zellen ist nicht nur diffus cytoplasmatisch, sondern auch extracellulär, und hier zwischen den Kollagenfibrillen, nachweisbar. Diese Befunde wurden diskutiert.
Notes:
Summary This report deals with a case of paraproteinemia (IgG Kappa) associated with skin indurations and yellowish papules on the trunk, edema of the face, and crystals in the cornea. In addition to the absence of elastic fibers and reduction of collagen fibers, light microscopy revealed single and/or multinucleated dermal cells with slight streaky patterning of the cytoplasm. These cells contained some sudanophilic (the greatest quantities of sudanophilic substances were observed in the deep corium) and partly birefringent material. In particular after warming, numerous spherocrystals appeared, which give Maltese cross birefringence. The presence of cholesterol esters can be assumed. The light-microscopic localization of acid phosphatase (incubation in Gomori medium) coincides in part with the distribution of birefringent lipids. Electron microscopy showed the cells with streaky contents to be chiefly histiocytes with numerous cristalline bodies in their cytoplasm. The cristalloids with a periodic inner structure are always surrounded by a single membrane. Identical bodies can be detected in endothelial cells of capillaries and epidermal keratinocytes. After pronase P incubation they disappear completely. We assume that the deposits correspond to phagocytized paraprotein. In addition, some histiocytic cells contain numerous lipid droplets in their cytoplasm. There is disintegration of some cells with crystalline inclusion, particularly in the deep corium. These cells show not only diffuse intracytoplasmic but also extracellular distribution of acid phosphatase activities. Particularly in the pericellular space of such cells numerous “banded structures” appear in connection with collagen degradation probably initiated by lysosomal enzymes. Amyloid could not be detected either light- or electron-microscopically.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00403803
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