Summary
Electron microscopic aspects in ten cases of normolipidemic cutaneous xanthomatosis have been investigated. Two additional types IV and V hyperlipoproteinaemic xanthomatosis have also been included.
Ultrastructural findings in all cases were similar. Abundant histiocytic cells with numerous intracytoplasmic lipid vacuoles, lysosomes, and myelin-figures, were the striking features. Moreover, in older lesions microfilaments and lipid vacuoles were found in some fibroblastic cells, as well as long space collagen around them. In some specimens we observed: giant multinucleated histiocytic cells, crystalline cleft-like spaces in histiocytes and some mastocytes with lipidic crystals in the extracellular space, as well as lipid vacuoles in Schwann cells, endothelial cells and pericytes. Rod-shaped tubulated bodies were found in some endothelial cells, with multiple basal vascular laminae. In xantelasma palpebrarum and in disseminate plane xanthoma the histiocytary foamy cells adopted a perivascular arrangement, as in hyperlipoproteinemic xanthomatosis.
We concluded that ultrastructural aspects of different xanthomatosis are fairly similar as a consequence of the large amount of intracytoplasmic lipids accumulated in xanthomatous cells. In xanthelasma palpebrarum and in disseminated plane xanthoma this cell phase is reached by similar pathways to those for hyperlipoproteinemic xanthomatosis, whilst in xanthoma disseminatum and juvenile xanthogranuloma the pathways seem to be different.
A classification of normolipidemic xanthomatosis is also provided.
Zusammenfassung
Eine elektronenmikroskopische Untersuchung von 10 Patienten mit normolipämischer Xanthomatose und von 2 Patienten mit hyperlipoproteinämischer Xanthomatose Typ IV und V ergab folgende Befunde:
In allen Fällen wurden große Mengen von Histiocyten mit intracytoplasmatischen Lipid-Vacuolen, Lysosomen und Myelinfiguren gesehen. Dazu waren Mikrofilamente und Lipid-Vacuolen in einigen Fibroblasten zu beobachten. In sämtlichen Präparaten fanden sich mehrkernige histiocytäre Riesenzellen mit kristallinen Spalträumen sowie einige Mastzellen. Lipid-Vacuolen konnten auch in Schwannzellen, Endothelzellen und in Pericyten nachgewiesen werden. In einigen Endothelzellen und Pericyten beobachtete man Duplikaturen der basalen vaskulären Laminae und “rob-shaped” tubuläre Körperchen. Beim Xanthelasma palpebrarum und dem Xanthoma planum disseminatum fanden sich die histiocytären Schaumzellen in perivaskulärer Anordnung, wie bei den hyperlipoproteinämischen Xanthomatosen.
Wir folgern, daß die ultrastrukturellen Aspekte in den verschiedenen Xanthomatosen im wesentlichen gleich sind, zumal große Ablagerungen von Fett in allen Zellen zu sehen sind. In den normolipämischen Xanthomatosen (Xanthelasma palpebrarum und Xanthoma planum disseminatum) ist die Art der Entstehung von Fettablagerungen vermutlich die gleiche wie bei den hyperlipoproteinämischen Xanthomatosen; sie unterscheiden sich jedoch von den Fettablagerungen beim Xanthoma disseminatum und beim Xanthogranuloma juvenile.
Eine Einteilung der normolipämischen Xanthomatosen wird vorgestellt.
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Ferrando, J., Bombí, J.A. Ultrastructural aspects of normolipidemic xanthomatosis. Arch Dermatol Res 266, 143–159 (1979). https://doi.org/10.1007/BF00694624
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DOI: https://doi.org/10.1007/BF00694624