Immunopathology of bullous pemphigoid, an autoimmune and inflammatory skin blistering disease

Keio Journal of Medicine

Volumn 52, Issue 2, 2003, Pages 128-133

  Liu, Zhi ^a  

^a UNIVERSITY OF NORTH CAROLINA SCHOOL OF MEDICINE   (United States)

Author keywords

Animal model; Bullous disease; Hemidesmosome

Indexed keywords

ANTIGEN; AUTOANTIBODY; CATHEPSIN G; CHEMOATTRACTANT; COLLAGENASE; COMPLEMENT; COMPLEMENT COMPONENT C5A; CYTOKINE; ELASTASE; EOSINOPHIL CATIONIC PROTEIN; EOTAXIN; GAMMA INTERFERON; GELATINASE; GELATINASE B; HISTAMINE; IMMUNOGLOBULIN G ANTIBODY; INTERLEUKIN 1; INTERLEUKIN 15; INTERLEUKIN 2; INTERLEUKIN 4; INTERLEUKIN 5; INTERLEUKIN 6; INTERLEUKIN 8; LEUKOTRIENE B4; MYELOPEROXIDASE; PROTEINASE; RANTES; REACTIVE OXYGEN METABOLITE; SCLEROPROTEIN; TUMOR NECROSIS FACTOR ALPHA;

CONFERENCE PAPER; DERMOEPIDERMAL JUNCTION; DISEASE SEVERITY; HEMIDESMOSOME; HUMAN; IMMUNOHISTOLOGY; IMMUNOPATHOGENESIS; IMMUNOPATHOLOGY; MAST CELL; NEUTROPHIL; NONHUMAN; PEMPHIGOID;

EID: 0037931462     PISSN: 00229717     EISSN: None     Source Type: Journal    
DOI: 10.2302/kjm.52.128     Document Type: Conference Paper

References (62)

1. Lever WF: Pemphigus. Medicine 1953; 32: 1-123
2. Stanley JR: Bullous pemphigoid. In: Fitzpatrick's Dermatology in General Medicine, Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz SI, editors. McGraw-Hill, New York, NY, 1999; 666-671
3. Jordon RE, Beutner EH, Witebsky E, Blumental G, Hale WL, Lever WF: Basement zone antibodies in bullous pemphigoid. JAMA 1967; 200: 751-756
4. Stanley JR, Hawley-Nelson P, Yuspa SH, Shevach EM, Katz SI: Characterization of bullous pemphigoid antigen: a unique basement membrane protein of stratified squamous epithelia. Cell 1981; 24: 897-903
5. Stanley JR, Woodley DT, Katz SI: Identification and partial characterization of pemphigoid antigen extracted from normal human skin. J Invest Dermatol 1984; 82: 108-111
6. Mutasim DF, Takahashi Y, Labib RS, Anhalt GJ, Patel HP, Diaz LA: A pool of bullous pemphigoid antigen(s) is intracellular and associated with the basal cell cytoskeleton-hemidesmosome complex. J Invest Dermatol 1985; 84: 47-53
7. Labib RS, Anhalt GJ, Patel HP, Mutasim DF, Diaz LA: Molecular heterogeneity of the bullous pemphigoid antigens as detected by immunoblotting. J Immunol 1986; 136: 1231-1235
8. Stanley JR, Tanaka T, Mueller S, Klaus-Kovtun V, Roop D: Isolation of complementary DNA for bullous pemphigoid antigen by use of patients' autoantibodies. J Clin Invest 1988; 82: 1864-1870
9. Diaz LA, Ratrie H 3rd, Saunders WS, Futamura S, Squiquera HL, Anhalt GJ, Giudice GJ: Isolation of a human epidermal cDNA corresponding to the 180-kD autoantigen recognized by bullous pemphigoid and herpes gestationis sera. Immunolocalization of this protein to the hemidesmosome. J Clin Invest 1990; 86: 1088-1094
10. Tanaka T, Parry DA, Klaus-Kovtun V, Steinert PM, Stanley JR: Comparison of molecularly cloned bullous pemphigoid antigen to desmoplakin I confirms that they define a new family of cell adhesion junction plaque proteins. J Biol Chem 1991; 266: 12555-12559
11. Sawamura D, Li K, Chu ML, Uitto J: Human bullous pemphigoid antigen (BPAG1). Amino acid sequences deduced from cloned cDNAs predict biologically important peptide segments and protein domains. J Biol Chem 1991; 266: 17784-17790
12. Giudice GJ, Emery DJ, Diaz LA: Cloning and primary structural analysis of the bullous pemphigoid autoantigen BP180. J Invest Dermatol 1992; 99: 243-250
13. Klatte DH, Kurpakus MA, Grelling KA, Jones JC: Immunochemical characterization of three components of the hemidesmosome and their expression in cultured epithelial cells. J Cell Biol 1989; 109: 3377-3390
14. Tanaka T, Korman NJ, Shimizu H, Eady RA, Klaus-Kovtun V, Cehrs K, Stanley JR: Production of rabbit antibodies against carboxy-terminal epitopes encoded by bullous pemphigoid cDNA. J Invest Dermatol 1990; 94: 617-623
15. Ishiko A, Shimizu H, Kikuchi A, Ebihara T, Hashimoto T, Nishikawa T: Human autoantibodies against the 230-kD bullous pemphigoid antigen (BPAG1) bind only to the intracellular domain of the hemidesmosome, whereas those against the 180-kD bullous pemphigoid antigen (BPAG2) bind along the plasma membrane of the hemidesmosome in normal human and swine skin. J Clin Invest 1993; 91: 1608-1615
16. Hopkinson SB, Riddelle KS, Jones JC: Cytoplasmic domain of the 180-kD bullous pemphigoid antigen, a hemidesmosomal component: molecular and cell biologic characterization. J Invest Dermatol 1992; 99: 264-270
17. Giudice GJ, Emery DJ, Zelickson BD, Anhalt GJ, Liu Z, Diaz LA: Bullous pemphigoid and herpes gestationis autoantibodies recognize a common non-collagenous site on the BP180 ectodomain. J Immunol 1993; 151: 5742-5750
18. Zillikens D, Rose PA, Balding SD, Liu Z, Olague-Marchan M, Diaz LA, Giudice GJ: Tight clustering of extracellular BP180 epitopes recognized by bullous pemphigoid autoantibodies. J Invest Dermatol 1997; 109: 573-579
19. Bernard P, Aucouturier P, Denis F, Bonnetblanc JM: Immunoblot analysis of IgG subclasses of circulating antibodies in bullous pemphigoid. Clin Immunol Immunopathol 1990; 54: 484-494
20. Dopp R, Schmidt E, Chimanovitch I, Leverkus M, Brocker EB, Zillikens D: IgG4 and IgE are the major immunoglobulins targeting the NC16A domain of BP180 in Bullous pemphigoid: serum levels of these immunoglobulins reflect disease activity. J Am Acad Dermatol 2000; 42: 577-583
21. Laffitte E, Skaria M, Jaunin F, Tamm K, Saurat JH, Favre B, Borradori L: Autoantibodies to the extracellular and intracellular domain of bullous pemphigoid 180, the putative key autoantigen in bullous pemphigoid, belong predominantly to the IgG1 and IgG4 subclasses. Br J Dermatol 2001; 144: 760-768
22. Haase C, Budinger L, Borradori L, Yee C, Merk HF, Yancey K, Hertl M: Detection of IgG autoantibodies in the sera of patients with bullous and gestational pemphigoid: ELISA studies utilizing a baculovirus-encoded form of bullous pemphigoid antigen 2. J Invest Dermatol 1998; 110: 282-286
23. Budinger L, Borradori L, Yee C, Eming R, Ferencik S, Grosse-Wilde H, Merk HF, Yancey K, Hertl M: Identification and characterization of autoreactive T cell responses to bullous pemphigoid antigen 2 in patients and healthy controls. J Clin Invest 1998; 102: 2082-2089
24. Lin MS, Fu CL, Giudice GJ, Olague-Marchan M, Lazaro AM, Stastny P, Diaz LA: Epitopes targeted by bullous pemphigoid T lymphocytes and autoantibodies map to the same sites on the bullous pemphigoid 180 ectodomain. J Invest Dermatol 2000; 115: 955-961
25. Baba T, Sonozaki H, Seki K, Uchiyama M, Ikesawa Y, Toriisu M: An eosinophil chemotactic factor present in blister fluids of bullous pemphigoid patients. J Immunol 1976; 116: 112-116
26. Czech W, Schaller J, Schopf E, Kapp A: Granulocyte activation in bullous diseases: release of granular proteins in bullous pemphigoid and pemphigus vulgaris. J Am Acad Dermatol 1993; 29: 210-215
27. Borrego L, Maynard B, Peterson EA, George T, Iglesias L, Peters MS, Newman W, Gleich GJ, Leiferman KM: Deposition of eosinophil granule proteins precedes blister formation in bullous pemphigoid. Comparison with neutrophil and mast cell granule proteins. Am J Pathol 1996; 148: 897-909
28. Diaz-Perez JL, Jordon RE: The complement system in bullous pemphigoid. IV. Chemotactic activity in blister fluid. Clin Immunol Immunopathol 1976; 5: 360-370
29. Katayama I, Doi T, Nishioka K: High histamine level in the blister fluid of bullous pemphigoid. Arch Dermatol Res 1984; 276: 126-127
30. Kawana S, Ueno A, Nishiyama S: Increased levels of immunoreactive leukotriene B4 in blister fluids of bullous pemphigoid patients and effects of a selective 5-lipoxygenase inhibitor on experimental skin lesions. Acta Derm Venereol 1990; 70: 281-285
31. D'Auria L, Cordiali Fei P, Ameglio F: Cytokines and bullous pemphigoid. Eur Cytokine Netw 1999; 10: 123-134
32. Wakugawa M, Nakamura K, Hino H, Toyama K, Hattori N, Okochi H, Yamada H, Hirai K, Tamaki K, Furue M: Elevated levels of eotaxin and interleukin-5 in blister fluid of bullous pemphigoid: correlation with tissue eosinophilia. Br J Dermatol 2000; 143: 112-116
33. Oikarinen AI, Zone JJ, Ahmed AR, Kiistala U, Uitto J: Demonstration of collagenase and elastase activities in the blister fluids from bullous skin diseases. Comparison between dermatitis herpetiformis and bullous pemphigoid. J Invest Dermatol 1983; 81: 261-266
34. Lauharanta J, Salonen EM, Vaheri A: Plasmin-like proteinase associated with high molecular weight complexes in blister fluid of bullous pemphigoid. Acta Derm Venereol 1989; 69: 527-529
35. Baird J, Lazarus GS, Belin D, Vassali JD, Busso N, Gubler P, Jensen PJ: mRNA for tissue-type plasminogen activator is present in lesional epidermis from patients with psoriasis, phemphigus or bullous pemphigoid, but is not detected in normal epidermis. J Invest Dermatol 1990; 95: 548-552
36. Gissler HM, Simon MM, Kraner MD: Enhanced association of plasminogen/plasmin with lesional epidermis of bullous pemphigoid. Br J Dermatol 1992;127: 272-277
37. Kramer MD, Reinartz J: The autoimmune blistering skin disease bullous pemphigoid. The presence of plasmin/alpha 2-antiplasmin complexes in skin blister fluid indicates plasmin generation in lesional skin. J Clin Invest 1993; 92: 978-983
38. Ståhle-Bäckdahl M, Inoue M, Guidice GJ, Parks WC: 92-kD gelatinase is produced by eosinophils at the site of blister formation in bullous pemphigoid and cleaves the extracellular domain of recombinant 180-kD bullous pemphigoid autoantigen. J Clin Invest 1994; 93: 2022-2030
39. Schaefer BM, Jaeger C, Drepper E, Kramer MD: Plasminogen activation in bullous pemphigoid immunohistology reveals urokinase type plasminogen activator, its receptor and plasminogen activator inhibitor type-2 in lesional epidermis. Autoimmunity 1996; 23: 155-164
40. Verraes S, Hornebeck W, Polette M, Borradori L, Bernard P: Respective contribution of neutrophil elastase and matrix metalloproteinase 9 in the degradation of BP180 (type XVII collagen) in human bullous pemphigoid. J Invest Dermatol 2001; 117: 1091-1096
41. Anhalt GJ, Labib RS, Voorhees JJ, Beals TF, Diaz LA: Induction of pemphigus in neonatal mice by passive transfer of IgG from patients with the disease. N Engl J Med 1982; 306: 1189-1196
42. Roscoe JT, Diaz L, Sampaio SA, Castro RM, Labib RS, Takahashi Y, Patel H, Anhalt GJ: Brazilian pemphigus foliaceus autoantibodies are pathogenic to BALB/c mice by passive transfer. J Invest Dermatol 1985; 85: 538-541
43. Liu Z, Diaz LA, Troy JL, Taylor AF, Emery DJ, Fairley JA, Giudice GJ: A passive transfer model of the organ-specific autoimmune disease, bullous pemphigoid, using antibodies generated against the hemidesmosomal antigen, BP180. J Clin Invest 1993; 92: 2480-2488
44. Chen R, Fairley JA, Zhao ML, Giudice GJ, Zillikens D, Diaz LA, Liu Z: Macrophages, but not T and B lymphocytes, are critical for subepidermal blister formation in experimental bullous pemphigoid: macrophage-mediated neutrophil infiltration depends on mast cell activation. J Immunol 2002; 169: 3987-3992
45. Liu Z, Roopenian DC, Zhou X, Christianson GJ, Diaz LA, Sedmak DD, Anderson CL: Beta2-microglobulin-deficient mice are resistant to bullous pemphigoid. J Exp Med 1997; 186: 777-783
46. Liu Z, Diaz LA, Swartz SJ, Troy JL, Fairley JA, Giudice GJ: Molecular mapping of a pathogenically relevant BP180 epitope associated with experimentally induced murine bullous pemphigoid. J Immunol 1995; 155: 5449-5454
47. Liu Z, Giudice GJ, Swartz SJ, Fairley JA, Till GO, Troy JL, Diaz LA: The role of complement in experimental bullous pemphigoid. J Clin Invest 1995; 95: 1539-1544
48. Chen R, Ning G, Zhao ML, Fleming MG, Diaz LA, Werb Z, Liu Z: Mast cells play a key role in neutrophil recruitment in experimental bullous pemphigoid. J Clin Invest 2001; 108: 1151-1158
49. Wintroub BU, Mihm MC Jr, Goetzl EJ, Soter NA, Austen KF: Morphologic and functional evidence for release of mast-cell products in bullous pemphigoid. N Engl J Med 1978; 298: 417-421
50. Liu Z, Giudice GJ, Zhou X, Swartz SJ, Troy JL, Fairley JA, Till GO, Diaz LA: A major role for neutrophils in experimental bullous pemphigoid. J Clin Invest 1997; 100: 1256-1263
51. Jordon RE, Kawana S, Fritz KA: Immunopathologic mechanisms in pemphigus and bullous pemphigoid. J Invest Dermatol 1985; 85 (1 Suppl): 72s-78s
52. Gammon WR: Immune complex and complement-mediated leukocyte recruitment in bullous pemphigoid. Immunol Ser 1989; 46: 509-525
53. Weiss SJ: Tissue destruction by neutrophils. N Engl J Med 1989; 320: 365-376
54. Liu Z, Shipley JM, Vu TH, Zhou X, Diaz LA, Werb Z, Senior RM: Gelatinase B-deficient mice are resistant to experimental bullous pemphigoid. J Exp Med 1998; 188: 475-482
55. Liu Z, Shapiro SD, Zhou X, Twining SS, Senior RM, Giudice GJ, Fairley JA, Diaz LA: A critical role for neutrophil elastase in experimental bullous pemphigoid. J Clin Invest 2000; 105: 113-123
56. Liu Z, Zhou X, Shapiro SD, Shipley JM, Twining SS, Diaz LA, Senior RM, Werb Z: The serpin alpha1-proteinase inhibitor is a critical substrate for gelatinase B/MMP-9 in vivo. Cell 2000; 102: 647-655
57. Yamamoto K, Inoue N, Masuda R, Fujimori A, Saito T, Imajoh-Ohmi S, Shinkai H, Sakiyama H: Cloning of hamster type XVII collagen cDNA, and pathogenesis of anti-type XVII collagen antibody and complement in hamster bullous pemphigoid. J Invest Dermatol 2002; 118: 485-492
58. Sitaru C, Schmidt E, Petermann S, Munteanu LS, Brocker EB, Zillikens D: Autoantibodies to bullous pemphigoid antigen 180 induce dermal-epidermal separation in cryosections of human skin. J Invest Dermatol 2002; 118: 664-671
59. Morrison LH, Labib RS, Zone JJ, Diaz LA, Anhalt GJ: Herpes gestationis autoantibodies recognize a 180-kD human epidermal antigen. J Clin Invest 1988; 81: 2023-2026
60. Bernard P, Prost C, Durepaire N, Basset-Seguin N, Didierjean L, Saurat JH: The major cicatricial pemphigoid antigen is a 180-kD protein that shows immunologic cross-reactivities with the bullous pemphigoid antigen. J Invest Dermatol 1992; 99: 174-179
61. Tamada Y, Yokochi K, Nitta Y, Ikeya T, Hara K, Owaribe K: Lichen planus pemphigoides: identification of 180 kd hemidesmosome antigen. J Am Acad Dermatol 1995; 32: 883-887
62. Zone JJ, Taylor TB, Meyer LJ, Petersen MJ: The 97 kDa linear IgA bullous disease antigen is identical to a portion of the extracellular domain of the 180 kDa bullous pemphigoid antigen, BPAg2. J Invest Dermatol 1998; 110: 207-210