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Familial Occurrence of a1-Antitrypsin Deficiency and Weber-Christian Disease

Familial Occurrence of a1-Antitrypsin Deficiency and Weber-Christian Disease Abstract • Severe panniculitis of the Weber-Christian type occurred in two brothers, both with marked α1-antitrypsin (α-AT) deficiency and phenotype PiZZ. Studies of inflammatory and immunologic function were undertaken in these two patients as well as in a third brother with severe α1-AT deficiency but without Weber-Christian disease. The findings of these investigations were suggestive of exaggerated immunologic and inflammatory function with enhanced lymphocyte responsiveness to phytohemagglutinin, enhanced activation of neutrophils and monocytes, and accelerated delayed hypersensitivity responses in all three subjects. This hyperreactivity may explain the apparent association of α1-AT deficiency with Weber-Christian disease. (Arch Dermatol 1983;119:198-202) References 1. Cox DW, Huber O: Rheumatoid arthritis and α1-antitrypsin . Lancet 1976;1:1216-1217. 2. Buisseret PD, Pembrey ME, Lessof MH: α1-Antitrypsin phenotypes in rheumatoid arthritis and ankylosing spondylitis . Lancet 1977;2:1358-1359.Crossref 3. Breit SN, Clark P, Penny R: α1-Protease inhibitor (α1-antitrypsin) phenotypes in rheumatic diseases, abstracted . Aust NZ J Med 1980;10:271.Crossref 4. Arnaud P, Galbraith RM, Faulk WP, et al: Increased frequency of the MZ phenotype of α1-protease inhibitor in juvenile chronic polyarthritis . J Clin Invest 1977;60:1442-1444.Crossref 5. Arnaud P, Chapuis-Cellier C, Souillet G, et al: High frequency of deficient Pi phenotypes of α1-antitrypsin in nonatopic infantile asthma . Trans Assoc Am Physicians 1976;89:205-214. 6. Geddes DM, Webley M, Brewerton DA, et al: α1-Antitrypsin phenotypes in fibrosing alveolitis and rheumatoid arthritis . Lancet 1977;2:1049-1050.Crossref 7. Brewerton DA, Webley M, Murphy AH, et al: The α1-antitrypsin phenotype MZ in acute anterior uveitis . Lancet 1978;1:1103.Crossref 8. Gewurz H, Svyehira LA: Complement , in Rose NR, Friedman H (eds): Manual of Clinical Immunology . American Society for Microbiology, 1976, pp 36-47. 9. Platts-Mills TAE, Ishizaka K: Activation of the alternate pathway of human complement by rabbit cells . J Immunol 1974;133:348-358. 10. Oppenheim JJ, Schechter B: Lymphocyte transformation , in Rose NR, Friedman H (eds): Manual of Clinical Immunology . American Society for Microbiology, 1976, pp 81-94. 11. Robinson JP, Penny R: Chemiluminescent response in normal human phagocytes: I. Automated measurements using a standard liquid scintillation counter . J Lab Clin Immunol 1982;7:215-217. 12. MacDonald A, Feiwel M: A review of the concept of Weber-Christian panniculitis with a report of five cases . Br J Dermatol 1968;80:355-361.Crossref 13. Milner RDG, Mitchinson MJ: Systemic Weber-Christian disease . J Clin Pathol 1965;18:150-156.Crossref 14. Kaneko F, Shunsuke I, Nagai M: A case of Weber-Christian disease suggesting cellular immunodeficiency . J Dermatol 1975;2:137-141. 15. Warter J, Storck D, Grosshans E, et al: Syndrome de Weber-Christian associe à un deficit en α1-antitrypsine . Ann Med Interne 1972;123:877-882. 16. Rubinstein HM, Joffer AM, Kudrna JC, et al: α1-Antitrypsin deficiency with severe panniculitis . Ann Intern Med 1977;86:742-744.Crossref 17. Bata J, Deviller P, Colobert L: Inhibition de la biosynthèse du DNA chez les lymphocytes humains par l'effet de la α1-antitrypsine . C R Acad Sci P 1977;285:1499-1501. 18. Arora PK, Miller HC, Aronson LD: α1-Antitrypsin is an effector of immunologic stasis . Nature 1977;274:589-590.Crossref 19. Vischer TL: Protease inhibitors reduce mitogen induced lymphocyte stimulation . Immunology 1979;36:811-813. 20. Fulton RJ, Hart DA: Detection and partial characterization of lymphoid cell surface proteases . Cell Immunol 1980;55:394-405.Crossref 21. Kaplan JG, Bona C: Proteases as mitogens . Exp Cell Res 1974;88:388-394.Crossref 22. Vischer TL, Bretz U, Baggiolini M: In vitro stimulation of lymphocytes by neutral proteases from human polymorphonuclear leukocyte granules . J Exp Med 1976;144:863-871Crossref 23. Lipsky JJ, Berninger RW, Hyman LR, et al: Presence of α1-antitrypsin on mitogen-stimulated human lymphocytes . J Immunol 1979;122:24-26. 24. Kitagawa S, Takaku F, Sakamoto S: Serine protease inhibitors inhibit superoxide production by human polymorphonuclear leukocytes and monocytes stimulated by various surface active agents . FEBS Lett 1979;107:331-334.Crossref 25. Nagai K, Nakamura T, Koyama J: Characterization of macrophage proteases involved in the ingestion of antigen-antibody complexes by the use of protease inhibitors . FEBS Lett 1978;92:299-302.Crossref 26. Goldstein BD, Witz G, Amoruso M, et al: Protease inhibitors antagonize the activation of polymorphonuclear leukocyte oxygen consumption . Biochem Biophys Res Commun 1979;88:854-860.Crossref 27. Kitagawa S, Takaku F, Sakamoto S: Evidence that proteases are involved in superoxide production by human polymorphonuclear leukocytes and monocytes . J Clin Invest 1980;65:74-81.Crossref 28. Musson RA, Becker EL: The role of an activatable esterase in immune dependent phagocytosis by human neutrophils . J Immunol 1977;118:1354-1364. 29. Dierich MP, Landen B, Schmitt M: Complement receptor analogous factors in human serum: Isolation of a molecule inhibitory for complement-dependent rosette formation, its identification as α1-antitrypsin and its functional characterization . Immunobiology 1979;156:153-167. 30. Mod A, Fust G, Gergely J, et al: Inhibition of C3-receptor-dependent phagocytosis by α1-antitrypsin , in Preud' Homme JL, Hawken VAL (eds): Proceedings of the Fourth International Congress of Immunology, 1980 . New York, Academic Press Inc, 1980, theme 11, workshop 4, paper 30. 31. Breit SN, Penny R: The role of α1-protease inhibitor (α1-antitrypsin) in the regulation of immunologic and inflammatory reactions . Aust NZ J Med 1980;10:449-453.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Familial Occurrence of a1-Antitrypsin Deficiency and Weber-Christian Disease

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References (35)

Publisher
American Medical Association
Copyright
Copyright © 1983 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1983.01650270016009
Publisher site
See Article on Publisher Site

Abstract

Abstract • Severe panniculitis of the Weber-Christian type occurred in two brothers, both with marked α1-antitrypsin (α-AT) deficiency and phenotype PiZZ. Studies of inflammatory and immunologic function were undertaken in these two patients as well as in a third brother with severe α1-AT deficiency but without Weber-Christian disease. The findings of these investigations were suggestive of exaggerated immunologic and inflammatory function with enhanced lymphocyte responsiveness to phytohemagglutinin, enhanced activation of neutrophils and monocytes, and accelerated delayed hypersensitivity responses in all three subjects. This hyperreactivity may explain the apparent association of α1-AT deficiency with Weber-Christian disease. (Arch Dermatol 1983;119:198-202) References 1. Cox DW, Huber O: Rheumatoid arthritis and α1-antitrypsin . Lancet 1976;1:1216-1217. 2. Buisseret PD, Pembrey ME, Lessof MH: α1-Antitrypsin phenotypes in rheumatoid arthritis and ankylosing spondylitis . Lancet 1977;2:1358-1359.Crossref 3. Breit SN, Clark P, Penny R: α1-Protease inhibitor (α1-antitrypsin) phenotypes in rheumatic diseases, abstracted . Aust NZ J Med 1980;10:271.Crossref 4. Arnaud P, Galbraith RM, Faulk WP, et al: Increased frequency of the MZ phenotype of α1-protease inhibitor in juvenile chronic polyarthritis . J Clin Invest 1977;60:1442-1444.Crossref 5. Arnaud P, Chapuis-Cellier C, Souillet G, et al: High frequency of deficient Pi phenotypes of α1-antitrypsin in nonatopic infantile asthma . Trans Assoc Am Physicians 1976;89:205-214. 6. Geddes DM, Webley M, Brewerton DA, et al: α1-Antitrypsin phenotypes in fibrosing alveolitis and rheumatoid arthritis . Lancet 1977;2:1049-1050.Crossref 7. Brewerton DA, Webley M, Murphy AH, et al: The α1-antitrypsin phenotype MZ in acute anterior uveitis . Lancet 1978;1:1103.Crossref 8. Gewurz H, Svyehira LA: Complement , in Rose NR, Friedman H (eds): Manual of Clinical Immunology . American Society for Microbiology, 1976, pp 36-47. 9. Platts-Mills TAE, Ishizaka K: Activation of the alternate pathway of human complement by rabbit cells . J Immunol 1974;133:348-358. 10. Oppenheim JJ, Schechter B: Lymphocyte transformation , in Rose NR, Friedman H (eds): Manual of Clinical Immunology . American Society for Microbiology, 1976, pp 81-94. 11. Robinson JP, Penny R: Chemiluminescent response in normal human phagocytes: I. Automated measurements using a standard liquid scintillation counter . J Lab Clin Immunol 1982;7:215-217. 12. MacDonald A, Feiwel M: A review of the concept of Weber-Christian panniculitis with a report of five cases . Br J Dermatol 1968;80:355-361.Crossref 13. Milner RDG, Mitchinson MJ: Systemic Weber-Christian disease . J Clin Pathol 1965;18:150-156.Crossref 14. Kaneko F, Shunsuke I, Nagai M: A case of Weber-Christian disease suggesting cellular immunodeficiency . J Dermatol 1975;2:137-141. 15. Warter J, Storck D, Grosshans E, et al: Syndrome de Weber-Christian associe à un deficit en α1-antitrypsine . Ann Med Interne 1972;123:877-882. 16. Rubinstein HM, Joffer AM, Kudrna JC, et al: α1-Antitrypsin deficiency with severe panniculitis . Ann Intern Med 1977;86:742-744.Crossref 17. Bata J, Deviller P, Colobert L: Inhibition de la biosynthèse du DNA chez les lymphocytes humains par l'effet de la α1-antitrypsine . C R Acad Sci P 1977;285:1499-1501. 18. Arora PK, Miller HC, Aronson LD: α1-Antitrypsin is an effector of immunologic stasis . Nature 1977;274:589-590.Crossref 19. Vischer TL: Protease inhibitors reduce mitogen induced lymphocyte stimulation . Immunology 1979;36:811-813. 20. Fulton RJ, Hart DA: Detection and partial characterization of lymphoid cell surface proteases . Cell Immunol 1980;55:394-405.Crossref 21. Kaplan JG, Bona C: Proteases as mitogens . Exp Cell Res 1974;88:388-394.Crossref 22. Vischer TL, Bretz U, Baggiolini M: In vitro stimulation of lymphocytes by neutral proteases from human polymorphonuclear leukocyte granules . J Exp Med 1976;144:863-871Crossref 23. Lipsky JJ, Berninger RW, Hyman LR, et al: Presence of α1-antitrypsin on mitogen-stimulated human lymphocytes . J Immunol 1979;122:24-26. 24. Kitagawa S, Takaku F, Sakamoto S: Serine protease inhibitors inhibit superoxide production by human polymorphonuclear leukocytes and monocytes stimulated by various surface active agents . FEBS Lett 1979;107:331-334.Crossref 25. Nagai K, Nakamura T, Koyama J: Characterization of macrophage proteases involved in the ingestion of antigen-antibody complexes by the use of protease inhibitors . FEBS Lett 1978;92:299-302.Crossref 26. Goldstein BD, Witz G, Amoruso M, et al: Protease inhibitors antagonize the activation of polymorphonuclear leukocyte oxygen consumption . Biochem Biophys Res Commun 1979;88:854-860.Crossref 27. Kitagawa S, Takaku F, Sakamoto S: Evidence that proteases are involved in superoxide production by human polymorphonuclear leukocytes and monocytes . J Clin Invest 1980;65:74-81.Crossref 28. Musson RA, Becker EL: The role of an activatable esterase in immune dependent phagocytosis by human neutrophils . J Immunol 1977;118:1354-1364. 29. Dierich MP, Landen B, Schmitt M: Complement receptor analogous factors in human serum: Isolation of a molecule inhibitory for complement-dependent rosette formation, its identification as α1-antitrypsin and its functional characterization . Immunobiology 1979;156:153-167. 30. Mod A, Fust G, Gergely J, et al: Inhibition of C3-receptor-dependent phagocytosis by α1-antitrypsin , in Preud' Homme JL, Hawken VAL (eds): Proceedings of the Fourth International Congress of Immunology, 1980 . New York, Academic Press Inc, 1980, theme 11, workshop 4, paper 30. 31. Breit SN, Penny R: The role of α1-protease inhibitor (α1-antitrypsin) in the regulation of immunologic and inflammatory reactions . Aust NZ J Med 1980;10:449-453.Crossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Mar 1, 1983

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