Ultrastructural aspects of normolipidemic xanthomatosis

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 xanthomatosus 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.