Skip to main content
SpringerLink
Log in

Zur Frage der Hautveränderungen bei einer Doppelparaproteinämie

Eine licht- und elektronenmikroskopische Untersuchung

On the question of cutaneous changes associated with paraproteinemia

Light- and electron-microscopic study

  • Original Contributions
  • Published:
Archives of Dermatological Research Aims and scope Submit manuscript

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.

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

Literatur

  1. Altman J, Winkelmann RK (1962) Diffuse normolipemic plane xanthoma. Arch Dermatol 85:633–640

    Google Scholar 

  2. Banfield WG, Lee CK, Lee CW (1973) Myocardial collagen of the fibrous long-spacing type. Arch Pathol 95:262–266

    Google Scholar 

  3. Barrett AJ (1969) Properties of lysosomal enzymes. In: Dingle JT, Fell HB (eds) Lysosomes, vol II. North-Holland Publ Comp, Amsterdam London, pp 245–312

    Google Scholar 

  4. Bazex A, Dupré A, Christol-Jalby B (1970) Xanthomatoses et dysglobulinémies. Bull Dermatol 77:607–620

    Google Scholar 

  5. Beutler StM, Fretzin DF, Jao W, Desser R (1978) Xanthomatosis resembling scleroderma in multiple myeloma. Arch Pathol 102:567–571

    Google Scholar 

  6. Bowen ID, Davies P (1971) The fine structural distribution of acid phosphatase in the digestive gland of arion hortensis (Fer.) Protoplasma 73:73–81

    Google Scholar 

  7. Braun-Falco O (1961) Die Histochemie der Haut. In: Gottron HA, Schönfeld W (Hrsg) Dermatologie und Venerologie, Bd I/1. Thieme, Stuttgart

    Google Scholar 

  8. Braun-Falco O, Rupec M (1964) Some observations on dermal collagen fibrils in ultrathin sections. J Invest Dermatol 42:15–19

    Google Scholar 

  9. Braun-Falco O, Rupec M (1967) Die Verteilung der sauren Phosphatase bei normaler und psoriatischer Verhornung (Eine elektronenmikroskopisch-cytochemische Untersuchung). Dermatologica 134:225–242

    Google Scholar 

  10. Charles CR, Cooper PhH, Helwig EB (1977) The fine structure of granuloma annulare. Lab Invest 36:444–451

    Google Scholar 

  11. Clyne DH, Brendstrup L, First MR, Pesce AJ, Finkel PN, Pollak VE, Pirani CL (1974) Renal effects of intraperitoneal kappa chain injection. Induction of crystals in renal tubular cells. Lab Invest 31:131–142

    Google Scholar 

  12. Danielsen L, Kobayasi T (1975) Ultrastructural changes in scleromyxedema. Acta Derm Venereol (Stockh) 55:451–460

    Google Scholar 

  13. Ferrando J, Bombi JA (1979) Ultrastructural aspects of normolipidemic xanthomatosis. Arch Dermatol Res 266:143–159

    Google Scholar 

  14. Finkel PN, Kronenberg K, Pesce AJ, Pollak VE, Pirani CL (1973) Adult fanconi syndrome, amyloidosis, and marked ⇂-light chain proteinuria. Nephron 10:1–24

    Google Scholar 

  15. Hashimoto K, Ohyama H (1974) Cross-bandes filamentous aggregation in the human dermis. J Invest Dermatol 62:106–112

    Google Scholar 

  16. Haust MD (1965) Fine fibrils of extracellular space (microfibrils). Am J Pathol 47:1113–1137

    Google Scholar 

  17. Ishikawa H, Maeda H, Takamatsu H, Saito Y (1979) Systemic hyalinosis (juvenile hyaline fibromatosis). Arch Dermatol Res 265:195–206

    Google Scholar 

  18. Jablońska S, Stachow A (1972) Scleroderma-like lesions in multiple myeloma. Dermatologica 144:257–269

    Google Scholar 

  19. Kahl FrR, Pearson RW (1967) Ultrastructural studies of experimental vesiculation. II. Collagenase. J Invest Dermatol 49:616–631

    Google Scholar 

  20. Kint A (1961) Les manifestations cutanées de la maladie de Kahler. Arch Belg Dermatol Syph 17:148–160

    Google Scholar 

  21. Klehr N, Heine H, Schaeg G, Nasemann Th (1977) Syntropie von Dermatochalasis und Urticaria pigmentosa als Folge einer Dermatosparaxis endoplasmatica. Hautarzt 28:522–534

    Google Scholar 

  22. Klein M, Grishman E (1977) Single cutaneous plasmacytoma with crystalloid inclusions. Arch Dermatol 113:64–68

    Google Scholar 

  23. Klintworth GK (1969) Experimental studies on the phagocytic capability of the corneal fibroblast. Am J Pathol 55:283–294

    Google Scholar 

  24. Klintworth GK, Bredehoeft StJ, Reed JW (1978) Analysis of corneal crystalline deposits in multiple myeloma. Am J Ophthalmol 86:303–313

    Google Scholar 

  25. Korting GW, Gilfrich HJ, Meyer zum Büschenfelde KH (1974) Scleroderma adultorum und Plasmocytom. Arch Dermatol Forsch 248:379–385

    Google Scholar 

  26. Koss MN, Pirani CL, Osserman EF (1976) Experimental Bence Jones cast nephropathy. Lab Invest 34:579–591

    Google Scholar 

  27. Lazarus GS, Brown RS, Daniels JR, Fullmer HM (1968) Zit. nach Reynolds JJ. Science NY 159:1483

    Google Scholar 

  28. Lloyd JB, Beck F (1974) Lysosomes. In: The cell in medical science, vol 1. Academic Press, London New York, p 273

    Google Scholar 

  29. Marien KJ, Smeenk G (1973) Generalised planar xanthomata associated with multiple myeloma and hyperlipoproteinemia. Arch Belg Dermatol Syph 29:317–318

    Google Scholar 

  30. Mohos StC, Wagner BM (1969) Damage to collagen in corneal immune injury. Arch Pathol 88:3–20

    Google Scholar 

  31. Mollo F, Monga G (1971) Banded structures in the connective tissue of lymphomas, lymphadenitis, and thymomas. Virchows Arch [Zell Pathol] 7:356–366

    Google Scholar 

  32. Monneron A, Bernhard W (1966) Action de certaines enzymes sur des tissus inclus en epon. J Microscopie 5:697–714

    Google Scholar 

  33. Moschella SL (1970) Plane xanthomatosis associated with myelomatosis. Arch Dermatol 101:683–687

    Google Scholar 

  34. Parakkal PF (1969) Involvement of macrophages in collagen resorption. J Cell Biol 41:345–354

    Google Scholar 

  35. Pinkerton MH, Robertson DM (1969) Corneal and conjunctival changes in dysproteinemia. Invest Ophthalmol 8:357–364

    Google Scholar 

  36. Reynolds JJ (1969) Connective tissue catabolism and the role of lysosomal enzymes. In: Dingle JT, Fell HB (eds) Lysosomes, vol II. North-Holland Publ Comp, Amsterdam London, pp 163–177

    Google Scholar 

  37. Rupec M (1967) Über irreguläre Korpuskeln mit kristalliner Innenstruktur in normalen menschlichen apokrinen Schweißdrüsen. Arch Klin Exp Dermatol 228:346–351

    Google Scholar 

  38. Rupec M (1970) Die Lysosomen. Z Haut Geschl Krh 45:67–83

    Google Scholar 

  39. Rupec M (1973) Die Ultrastruktur der Epidermis. In: Doerr W, Seifert G, Uehlinger E (Hrsg) Spezielle pathologische Anatomie, Bd 7. Springer, Berlin Heidelberg New York, S 691

    Google Scholar 

  40. Rupec M (1977) Zur Ultrastruktur und sauren Phosphatase-Aktivität in den Schweißdrüsen mit “clear, reticulated cytoplasm”. Arch Dermatol Res 258:193–201

    Google Scholar 

  41. Rupec M (im Druck) Mikroskopische und elektronenmikroskopische Anatomie der Haut. In: Korting GW (Hrsg) Dermatologie in Praxis und Klinik, Bd I. Thieme, Stuttgart

  42. Rupec M, Brühl R (1976) On the question of the ultrastructure and composition of corpus intra cristam after local methylcholanthrene application. J Ultrastruct Res 54:388–396

    Google Scholar 

  43. Shelley WB, Rawnsley HM (1975) Plasma cell granulomas in non-lipemic xanthomatosis: Apparent induction by indomethacin. Acta Derm Venereol (Stockh) 55:489–492

    Google Scholar 

  44. Sun CN, White HJ (1975) Extracellular cross-striated banded structures in human connective tissue. Tissue Cell 7:419–432

    Google Scholar 

  45. Schin KS, Clever E (1965) Lysosomal and free acid phosphatase in salivary glands of chironomus tentans. Science 150:1053–1055

    Google Scholar 

  46. Schnyder UW, Kaufmann J (1979) Demonstrationen. Dermatologica 159:407–412

    Google Scholar 

  47. Schwarz W, Güldner FH (1967) Elektronenmikroskopische Untersuchungen des Kollagenabbaus im Uterus der Ratte nach der Schwangerschaft. Z Zellforsch 83:416–426

    Google Scholar 

  48. Stadler R, Orfanos CE (1978) Reifung und Alterung der elastischen Fasern. Arch Dermatol Res 262:97–111

    Google Scholar 

  49. Welsh RA, Meyer AT (1967) Intracellular collagen fibers. Arch Pathol 84:354–362

    Google Scholar 

  50. Woods JF, Nichols G, Jr (1965): Collagenolytic activity in rat bone cells. J Cell Biol 26:747–757

    Google Scholar 

  51. Wynn CH (1967) Solubilization of insoluble collagens by rat liver lysosomes. Nature 215:1191–1192

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Hautklinik der Philipps-Universität, Deutschhausstr. 9, D-3550, Marburg/Lahn, Bundesrepublik Deutschland

    M. Rupec

  2. Medizinische Klinik der Philipps-Universität, D-3550, Marburg/Lahn

    K. Havemann

  3. Augenklinik, Stadtkrankenhaus, D-3500, Kassel

    W. Aust

  4. Hautklinik, Stadtkrankenhaus, D-3500, Kassel

    K. Wulf

Authors
  1. M. Rupec
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. K. Havemann
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. W. Aust
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. K. Wulf
    View author publications

    You can also search for this author in PubMed Google Scholar

Additional information

Herrn Prof. Dr. W. F. Lever zum 70. Geburtstag gewidmet

Mit Unterstützung der Deutschen Forschungsgemeinschaft

Rights and permissions

Reprints and permissions

About this article

Cite this article

Rupec, M., Havemann, K., Aust, W. et al. Zur Frage der Hautveränderungen bei einer Doppelparaproteinämie. Arch Dermatol Res 268, 191–206 (1980). https://doi.org/10.1007/BF00403803

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00403803

Key words

Schlüsselwörter

Search

Navigation