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Auto-immune mechanisms as a probable aetiology of Behçet's syndrome, an electron microscopic study of the oral mucosa

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Summary

A constant and characteristic feature of Behçet's syndrome was the association of macrophages with degenerated prickle cells in the prickle-cell layer of the oral mucosa adjacent to ulcerations. Three types of macrophages could be identified: Thetype I macrophage, rich in cytoplasmic organelles, phagocytosed material from degenerated cells. Thetype II macrophages with scant endoplasmic reticulum came in contact with small lymphocytes which they gave immunological information, inducing them to undergo blastoid transformation. These lymphoblasts produced immunoglobulins. Lymphoid cells that contained numerous ribosomes and polysomes were attached to the prickle cells and probably elaborated a cytotoxic factor, since the cytoplasm of the prickle cells ultimately degenerated and the ulcer expanded. The cytoplasm of prickle cells apparently acts as an auto-antigen. Immune responses against it are mediated by the macrophages and the lymphoid cells. The changes seen are consistent with those of delayed hypersensitivity reactions.Type III macrophages had Birbeck granules and were regarded as Langerhans cells. Thymus-dependenttype III macrophages may have an important role in developing and controlling the ulceration in Behçet's syndrome. The involvement of lysosomes in initiating the ulceration is discussed.

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References

  • Able, M. E., Lee, J. C., Rosenau, W.: Lymphocyte-target cell interactionin vitro. Amer. J. Path.60, 421–434 (1970).

    Google Scholar 

  • Austen, K. F., Cohn, Z. A.: Contribution of serum and cellular factors in host defense reactions. I. Serum factors in host resistance. New Engl. J. Med.268, 933–938; 994–1000 (1963).

    Google Scholar 

  • Behçet, H.: Über rezidivierende, apthöse, durch ein Virus verursachte Geschwüre am Mund, am Auge und an den Genitialien. Derm. Wschr.105, 1152–1157 (1937).

    Google Scholar 

  • Birbeck, M. S., Breathnach, A. S., Everall, J. D.: Melanocytes and high-level clear cells (Langerhans cells) in vitiligo. J. invest. Derm.37, 51–64 (1961).

    Google Scholar 

  • Breathnach, A. S., Wyllie, L. M.: Electron microscopy of melanocytes and Langerhans cells in human fetal epidermis at fourteen weeks. J. invest. Derm.44, 51–60 (1965).

    Google Scholar 

  • Kuwahara, H.: Electron microscopic studies on dendritic cells of the epidermis, especially on relation between melanocytes and Langerhans cells. Jap. J. Derm.79, 620–650 (1969).

    Google Scholar 

  • —— Electron microscopic studies on dendritic cells of the epidermis, especially on the function of Langerhans cells and etiology of certain leucodermas. Jap. J. Derm.80, 69–112 (1970).

    Google Scholar 

  • Lehner, T.: Autoimmunity and management of recurrent oral ulceration. Brit. dent. J.122, 15–20 (1967a).

    Google Scholar 

  • —— Behçet's syndrome and autoimmunity. Brit. med. J.1967bI, 465–467.

    Google Scholar 

  • —— Characterization of mucosal antibodies in recurrent aphthous ulceration and Behçet's syndrome. Arch. oral Biol.14, 843–853 (1969a).

    Google Scholar 

  • —— Pathology of recurrent oral ulceration and oral ulceration in Behçet's syndrome. Light, electron and fluorescence microscopy. J. Path. Bact.97, 481–494 (1969b).

    Google Scholar 

  • Lepow, I. H.: In: Teichman, N. S., Mediation on inflammation by the polymorphonuclear leucocyte as a sequela of immune reactions (general discussion, p 232). J. Periodont.41, 228–239 (1970).

    Google Scholar 

  • Listgarten, M. A.: The ultrastructure of human gingival epithelium. Amer. J. Anat.114, 49–69 (1964).

    Google Scholar 

  • Luft, J. H.: Improvements in epoxy resin embedding methods. J. biophys. biochem. Cytol.9, 409–414 (1961).

    Google Scholar 

  • Müller-Eberhard, H. J.: Chemistry and reaction mechanisms of complement. Advanc. Immunol.8, 1–80 (1968).

    Google Scholar 

  • Nettesheim, P., Makinodan, T.: Differentiation of lymphocytes undergoing an immune response in diffusion chambers. J. Immunol.94, 868–876 (1965).

    Google Scholar 

  • Nishiyama, S. Klinische Beobachtungen über Behçet'scher Krankheit. Jap. J. Derm.69, 1139–1185 (1959).

    Google Scholar 

  • Odland, G. F.: A submicroscopic granular component in human epidermis. J. invest. Derm.34, 11–15 (1960).

    Google Scholar 

  • Osawa, N., Saito, K., Mitsuhashi, S.: Transfer agent of immunity. V. Antibody formation of cultured macrophages treated with an immune ribonucleic acid preparation. Jap. J. Microbiol.15, 29–37 (1971).

    Google Scholar 

  • Oshima, Y., Shimizu, T., Yokohari, R., Matsumoto, T., Kano, K., Kagami, T., Nagaya, H.: Clinical studies on Behçet's syndrome. Ann. rheum. Dis.22, 36–45 (1963).

    Google Scholar 

  • Sato, S.: Histological and electron microscopic studies of the dendritic cells in human oral epithelium. Jap. J. Derm.80, 585–609 (1970).

    Google Scholar 

  • Sato, T., Kikuchi, M, Hirari, T., Saigo, K., Suzuki, K., Honma, T.: Immunological study on the diseases of oral mucosa: Serum protein patterns by immuno-electrophoresis. Jap. J. Periodont.12, 3–6 (1970).

    Google Scholar 

  • Schoenberg, M. D., Mumaw, V. R., Moore, R. D., Weisberger, A. S.: Cytoplasmic interaction between macrophages and lymphocytic cells in antibody synthesis. Science143, 964–965 (1964).

    Google Scholar 

  • Shikano, S.: Histopathological study on Behçet's disease. Jap. J. Ophthal.64, 2341–2368 (1960).

    Google Scholar 

  • Shimizu, T., Katsuta, Y., Oshima, Y.: Immunological studies on Behçet's syndrome. Ann. rheum. Dis.24, 494–500 (1965).

    Google Scholar 

  • Waterhouse, J. P., Squier, C. A.: The Langerhans cell in human gingival epithelium. Arch. oral Biol.12, 341–348 (1967).

    Google Scholar 

  • Wiener, J., Spiro, D., Russell, P. S.: An electron microscopic study of the homograft reaction. Amer. J. Path.44, 319–347 (1964).

    Google Scholar 

  • Wolff, K., Holubar, K.: Odland-Körper (membrane-coating granules, Kerationosomen als epidermale Lysosomen. Arch. klin. exp. Derm.231, 1–19 (1967).

    Google Scholar 

  • Zelickson, A. S.: The Langerhans cell. J. invest. Derm.44, 201–212 (1965).

    Google Scholar 

  • —— Hartmann, J. F.: An electron microscope study of normal human non-keratinizing oral mucosa. J. invest. Derm.38, 99–107 (1962).

    Google Scholar 

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A preliminary report was given at the 6th Asian-Pacific Dental Congress in Bangkok, Thailand. September 18–21, 1970.

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Saito, T., Honma, T., Sato, T. et al. Auto-immune mechanisms as a probable aetiology of Behçet's syndrome, an electron microscopic study of the oral mucosa. Virchows Arch. Abt. A Path. Anat. 353, 261–272 (1971). https://doi.org/10.1007/BF00545734

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  • DOI: https://doi.org/10.1007/BF00545734

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