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Ultrastructural aspects of normolipidemic xanthomatosis

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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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References

  • Abramov, A., Schorr, S., Wolman, M.: Generalized xanthomatosis with calcified adrenals. Am. J. Dis. Child.91, 282–286 (1956)

    Google Scholar 

  • Adamson, H. G.: Congenital xanthoma multiplex in child. Br. J. Dermatol.17, 222 (1905)

    Google Scholar 

  • Altman, J., Winkelmann, R. K.: Diffuse normolipemic plane xanthoma. Arch. Dermatol.85, 633–640 (1962)

    Google Scholar 

  • Aubert, L., Arroyo, H., Detoll, P.: Etude clinique et cytologique d'un deuxième cas d'IgA-myélome xanthomateux avec auto-anticorps circulant antilipoproteine. Semaine des Hôpitaux de Paris43, 3014–3021 (1967)

    Google Scholar 

  • Balas, O., Bazex, J., Tabet, G., Broussy, J., Bazex, A.: Etude histochimique et ultrastructurale de la peau normale de sujets atteints d'hyperlipémie type IV. Ann. Dermatol. Syph.102, 423–428 (1975)

    Google Scholar 

  • Beaumont, J. L., Jacotot, B., Vilain, C., Beaumont, V.: Myélome, hyperlipidémie et xanthomatose. Nouvelle revue française d'hematologie and blood cells5, 507–517 (1965)

    Google Scholar 

  • Bhattacharyya, A. K., Connor, W. E.: Beta-sitosterolemia and xanthomatosis. A newly described lipid storage disease in two sisters. J. Clin. Invest.53, 1033–1044 (1974)

    Google Scholar 

  • Bogaert, L. van, Scherer, H. J., Epstein, E.: Une forme cérebrale de la cholesterinose generalisée (ed.) Paris: Masson 1937

    Google Scholar 

  • Braun-Falco, O.: Struktur und Morphogenese von Xanthomen bei Hyperlipoproteinämie vom Typ III. Hautarzt27, 122–132 (1976)

    Google Scholar 

  • Denys, P., Malbrain, H.: Contribution à l'étude des réticulo-endothélioses de l'enfant (la xantholeucémie). Arch. Franç. Pediat.11, 707–716 (1954)

    Google Scholar 

  • Endo (1910): Quoted by Feyrter. Virch. Arch. für Pathol. Anat. Physiol. Klinische Med.273, 736–741 (1929)

    Google Scholar 

  • Ericsson, J. L. E., Biberfeld, P.: Studies on aldehyde fixation. Fixation rates and their relation to fine structure and some histochemical reactions in liver. Lab. Invest.17, 281–298 (1967)

    Google Scholar 

  • Esterly, N. B., Sahihi, T., Medenica, M.: Juvenile xanthogranuloma. An atypical case with study of ultrastructure. Arch. Dermatol.105, 99–102 (1972)

    Google Scholar 

  • Ferrando, J., Corominas, A., Piñol Aguade, J., Grimalt, F., Castells Rodellas, A.: Estudios con cromatografia de lípidos en piel normal y en diferentes tipos de xantomatosis (nota previa). Med. Cutánea7, 349–358 (1973)

    Google Scholar 

  • Ferrando, J., Corominas Vilardell, A.: Estudio de la composición lipídica de las lesiones cutáneas en diferentes tipos de xantomatosis. Med. Cut. Ibero Lat. Am.5, 415–424 (1977)

    Google Scholar 

  • Ferrando, J., Lecha, M., Estrach, T., Piñol Aguade, J.: Xantoma plano diseminado con fotosensibilidad. Med. Cut. Iber Lat. Am.3, 23–28 (1975)

    Google Scholar 

  • Ferrans, V. J., Fredrickson, D. S.: The pathology of Tangier disease. A light and electron microscopic study. Am. J. Pathol.78, 101–123 (1975)

    Google Scholar 

  • Fredrickson, D. S., Levy, R. J., Lees, R. S.: Fat transport in lipoproteins: An integrated approach to mechanisms and disorders. N. Engl. J. Med.276, 34–44, 94–103, 148–156, 215–226, 237–281 (1976)

    Google Scholar 

  • Ghadially, F. N.: Ultrastructural pathology of the cell. London: Butterworth 1975

    Google Scholar 

  • Gonzalez-Crussi, F., Campbell, R. J.: Juvenile xanthogranuloma, ultrastructural study. Arch. Pathol.89, 65–72 (1970)

    Google Scholar 

  • Grafe, J. von: Verhandlungen ärztlicher Gesellschaften: Ein Fall von eigentümlichen Tumoren auf Hornhäuten, Klin. Wochenschr.4, 323–329 (1867)

    Google Scholar 

  • Harkin, J. C., Reed, R. J.: Tumors of the peripheral nervous system. Armed Forces Inst. Pathol., p. 43. Washington 1969

  • Hasegawa, T., Uyeda, K.: Ultrastructure of xanthelasma palpebrarum as revealed by freeze-etching techniques. Arch. Dermatol. Forsch.248, 347–354 (1974)

    Google Scholar 

  • Helwig, E. B., Hackney, V. C.: Juvenile xanthogranuloma (nevoxanthoendothelioma). Am. J. Pathol.30, 625–626 (1954)

    Google Scholar 

  • Hu, Ch-H, Winkelmann, R. K.: Unusual normolipemic cutaneous xanthomatosis. A comparison of two cases illustrating the differential diagnosis. Acta Derm. Venereol. (Stockh.)57, 421–429 (1977)

    Google Scholar 

  • Imaeda, T.: Electron-microscopic study of xanthoma cells. J. Invest. Dermatol.34, 331–337 (1960)

    Google Scholar 

  • Kametani, S.: Xanthoma of the stomach. Gastrointest. Endosc.5, 34–37 (1963)

    Google Scholar 

  • Kjaerheim, A., Stokke, T.: Juvenile xanthogranuloma of the oral cavity. Oral Surg.38, 414–425 (1974)

    Google Scholar 

  • Kodama, H., Nakagawa, S., Tanioku, R.: Plane xanthomatosis with antilipoprotein autoantibody. Arch. Dermatol.105, 722–727 (1972)

    Google Scholar 

  • Lemeunier, A., Burri, P. H., Weibel, E. R.: Absence of acid phosphatase activity in specific endothelial organelles. Histochemie20, 143–149 (1969)

    Google Scholar 

  • MacDonach, J. E. R.: A contribution to our knowledge of the naevoxantho-endotheliomata. Br. J. Dermatol.24, 85–99 (1912)

    Google Scholar 

  • Marien, K. J. C., Smeenk, G.: Plane xanthomata associated with multiple myeloma and hyperlipoproteinaemia. Br. J. Dermatol.93, 407–415 (1975)

    Google Scholar 

  • Miller, A. S., Elzay, R. P.: Verruciform xanthoma of the gingiva: report of six cases. J. Periodontol.44, 103–105 (1973)

    Google Scholar 

  • Parker, F., Odland, G. G.: Experimental xanthoma. Acorrelative biochemical, histologic, histochemical, and electron-microscopic study. Am. J. Pathol.53, 537–565 (1968)

    Google Scholar 

  • Parker, F., Short, J. M.: Xanthomatosis associated with hyperlipoproteinemia. J. Invest. Dermatol.55, 71–88 (1970)

    Google Scholar 

  • Perrot, H., Thivolet, J., Hermier, C., Ortonne, J.-P.: Xanthoma disseminatum aspect ultrastructural d'histiocytosis X. Bulletin de la Société Française de Dermatol. Syph.79, 674–675 (1972)

    Google Scholar 

  • Piñol Aguade, J., Ferrando, J., Bombi, J. A., Castel, T., Lecha, M., Ferrandiz, C., Fontarnau, R.: Melanoma desmoplástico. Med. Cut. Ibero Lat. Am.5, 77–92 (1977)

    Google Scholar 

  • Piñol Aguade, J., Ferrando, J., Castells Mas, A., Castel, T., Castells Rodellas A., Fernandez, N., Corominas, A.: Sobre la posición nosológica y el estado inmunitario del “Xanthoma disseminatum”. Med. Cut.7, 9–26 (1973)

    Google Scholar 

  • Possick, P.: Plasma cells dyscrasia (monoclonal gammopathy) associated with diffuse normolipemic plane xanthomatosis. Basal cell epitheliomatosis. Arch. Dermatol.100, 252–254 (1969)

    Google Scholar 

  • Queyrat, L., Laroche, G.: Xanthome plan généralisé. Bulletin de la Société Française de Dermatologie et Syphiligraphie27, 208–211 (1920)

    Google Scholar 

  • Rayer, P. F. O.: Traité théorique et pratique des maladies de la peau. Avec un Atlas, 2nd (ed.) Vol. III. Paris: J.-B. Baillière et fils 1835

    Google Scholar 

  • Rosshoff, W., Kratka, J., Orfanos, C. E.: Multinoduläre Retikulohistiozytose des Erwachsenen. Hautarzt29, 578–582 (1978)

    Google Scholar 

  • Richardson, K. C., Jarett, L., Finke, E. H.: Embedding in epoxy resins for ultrathin sectioning in electron microscopy. Stain Technol.35, 313–323 (1960)

    Google Scholar 

  • Sandbank, M.: Cross-striated banded structures in cutaneous leishmaniasis. Br. J. Dermatol.96, 653–657 (1977)

    Google Scholar 

  • Shafer, W. G.: Verruciform xanthoma. Oral Surg.31, 784–789 (1971)

    Google Scholar 

  • Shehbens, W. E., Ludatscher, R. M.: Fine structure of senile angiomas of human skin. Angiology19, 581–592 (1968)

    Google Scholar 

  • Stevanovic, D., Lalevic, B., Majcan, D.: Xanthomes disséminés. Une forme cutanée de la maladie de Hand-Schüller-Christian. Journaux Dermatologiques de Paris (9th and 10th March 1977) Société Française de Dermatologie et Synphiligraphie

  • Tannhauser, S. J.: Lipidoses, 2nd. ed. Oxford Med. Publ. 1950

  • Thelander, H. E., Grane, J. J., Lanmann, J. T.: Xanthomatosis. J. Pediatr34, 490–496 (1949)

    Google Scholar 

  • Virchow (1957): Quoted by Ghadially, In: Ultrastructural pathology of the cell. London: Butterworth (1975)

    Google Scholar 

  • Weibel, E. R., Palade, G. E.: New cytoplasmic components in arterial endothelia. J. Cell Biol.23, 101–112 (1964)

    Google Scholar 

  • White, J. G., Clawson, G. G.: Blood cells and blood vessels. In: Ultrastructure of normal and abnormal skin, (ed.) Zelickson, A. S., p. 261. Philadelphia Lea and Febiger 1967

    Google Scholar 

  • Wilson (1863): Quoted by Braun-Falco. Natr. Metabol.15, 68–88 (1973)

    Google Scholar 

  • Wolman, M., Sterk, V. V., Gatt, S., Frenkel, M.: Primary familial xanthomatosis with involvement and calcification of the adrenals. Pedriatrics28, 742–757 (1961)

    Google Scholar 

  • Zegarelli, D. J., Zegarelli-Schmidt, E. C., Zegarelli, E. V.: Verruciform xanthoma. A clinical, light microscopic, and electron microscopic study of two cases. Oral Surg.38, 725–734 (1974)

    Google Scholar 

  • Zemal, H., Deeken, J., Asel, N., Packer, J.: The ultrastructural features of normolipemic plane xanthoma. Arch. of Pathol.89, 111–117 (1970)

    Google Scholar 

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Authors and Affiliations

  1. Department of Dermatology (Prof. J. M. Mascaró), Faculty of Medicine, University of Barcelona, c/Casanova 143, 36, Barcelona, Spain

    J. Ferrando

  2. Department of Pathology (Prof. D. Ribas Mujal), Faculty of Medicine, University of Barcelona, c/Casanova 143, 36, Barcelona, Spain

    J. A. Bombí

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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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