Skip to main content
SpringerLink
Log in

Bullous Pemphigoid

From Bench to Bedside

  • Therapy In Practice
  • Published:
Drugs Aims and scope Submit manuscript

Abstract

Bullous pemphigoid (BP) is a chronic, autoimmune, blistering disease observed primarily in the elderly population. Several clinical variants have been described, including classic (bullous), localised, nodular, vegetating, erythrodermic, erosive, childhood and drug-induced forms. Autoantibodies target the BP230 and BP180 antigens, located in the hemidesmosomal complex of the skin basement membrane zone. Subsequent complement activation recruits chemical and cellular immune mediators to the skin, ultimately resulting in blister formation. Both autoantibodies and complement may be detected by various immunofluorescent, immune electron microscopy and molecular biology techniques. Recent trials suggest that potent topical corticosteroids should be considered as first-line therapy. Tetracycline with or without nicotinamide may benefit a subset of patients with mild BP. Oral corticosteroids should rarely exceed 0.75 mg/kg/day and corticosteroid-sparing agents may be useful for recalcitrant disease.

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

Fig. 1
Table I
Table II
Fig. 2
Table III
Table IV

Similar content being viewed by others

References

  1. Bernard P, Vaillant L, Labeille B, et al. Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Arch Dermatol 1995; 131: 48–52

    Article  PubMed  CAS  Google Scholar 

  2. Zillikens D, Wever S, Roth A, et al. Incidence of autoimmune subepidermal blistering dermatoses in a region of Central Germany. Arch Dermatol 1995; 131: 957–8

    Article  PubMed  CAS  Google Scholar 

  3. Jung M, Kippes W, Messer G, et al. Increased risk of bullous pemphigoid in male and very old patients: a population-based study on incidence. J Am Acad Dermatol 1999; 41: 266–8

    Article  PubMed  CAS  Google Scholar 

  4. Korman NJ. Bullous pemphigoid. J Am Acad Dermatol 1987; 16: 907–24

    Article  PubMed  CAS  Google Scholar 

  5. Lever WF. Pemphigus and pemphigoid. J Am Acad Dermatol 1979; 1: 2–31

    Article  PubMed  CAS  Google Scholar 

  6. Venning VA, Wojnarowska F. Lack of predictive factors for the clinical course of bullous pemphigoid. J Am Acad Dermatol 1992; 26: 585–9

    Article  PubMed  CAS  Google Scholar 

  7. Korman NJ. Bullous pemphigoid: the latest in diagnosis, prognosis and therapy. Arch Dermatol 1998; 134: 1137–41

    Article  PubMed  CAS  Google Scholar 

  8. Savin JA. Some factors affecting prognosis in pemphigus vulgaris and pemphigoid. Br J Dermatol 1981; 104: 415–20

    Article  PubMed  CAS  Google Scholar 

  9. Roujeau JC, Lok C, Bastuji-Garin S, et al. High risk of death in elderly patients with extensive bullous pemphigoid. Arch Dermatol 1998; 134: 465–9

    Article  PubMed  CAS  Google Scholar 

  10. Rzany B, Partscht K, Jung M, et al. Risk factors for lethal outcome in patients with bullous pemphigoid. Arch Dermatol 2002; 138: 903–8

    Article  PubMed  Google Scholar 

  11. Ahmed AR, Maize JC, Provost TT. Bullous pemphigoid. Arch Dermatol 1977; 113: 1043–6

    Article  PubMed  CAS  Google Scholar 

  12. Taylor G, Venning VA, Wojnarowska FT, et al. Bullous pemphigoid and autoimmunity. J Am Acad Dermatol 1993; 29: 181–4

    Article  PubMed  CAS  Google Scholar 

  13. Delgado JC, Turbay D, Yunis EJ, et al. A common major histocompatibility complex class II allele HLA-DQB*0301 is present in clinical variants of pemphigoid. Proc Natl Acad Sci U S A 1996; 93: 8569–71

    Article  PubMed  CAS  Google Scholar 

  14. Banfield CC, Wojnarowska F, Allen J, et al. The association of HLA-DQ7 with bullous pemphigoid is restricted to men. Br J Dermatol 1998; 138: 1085–90

    Article  PubMed  CAS  Google Scholar 

  15. Budinger L, Borradori L, Yee C, et al. Identification and characterization of autoreactive T cell responses to bullous pemphigoid antigen 2 in patients and healthy controls. J Clin Invest 1998; 102: 2082–9

    Article  PubMed  CAS  Google Scholar 

  16. Schaller J, Feleke W, Haustein UF, et al. HLA in bullous pemphigoid: the probable role of HLA-B7 as a marker for poor responders to immunosuppressive therapy. Int J Dermatol 1991; 30: 36–8

    Article  PubMed  CAS  Google Scholar 

  17. Giannini JM, Callen JP, Gruber GG. Bullous pemphigoid and rheumatoid arthritis. J Am Acad Dermatol 1981; 4: 695–7

    Article  PubMed  CAS  Google Scholar 

  18. Clayton CA, Burnham TK. Systemic lupus erythematosus and coexisting bullous pemphigoid: immunofluorescent investigations. J Am Acad Dermatol 1982; 7: 236–45

    Article  PubMed  CAS  Google Scholar 

  19. Stoll DM, King LE. Association of bullous pemphigoid with systemic lupus erythematosus. Arch Dermatol 1984; 120: 362–6

    Article  PubMed  CAS  Google Scholar 

  20. Huang CY, Chen TC. Bullous pemphigoid associated with systemic lupus erythematosus: the discrimination of antibasement membrane zone antibody. Int J Dermatol 1997; 36: 40–2

    Article  PubMed  CAS  Google Scholar 

  21. Goihman-Yahr M. Familial occurrence of coexistence of bullous pemphigoid and Sjogren’s syndrome. Int J Dermatol 1998; 37: 475–6

    Google Scholar 

  22. Masouye I, Schmied E, Didierjean L, et al. Bullous pemphigoid and multiple sclerosis: more than a coincidence? J Am Acad Dermatol 1989; 21: 63–8

    Article  PubMed  CAS  Google Scholar 

  23. James WD. Bullous pemphigoid, myasthenia gravis, and thymoma. Arch Dermatol 1984; 120(3): 397

    Article  PubMed  CAS  Google Scholar 

  24. Hamilton DV, McKenzie AW. Bullous pemphigoid and primary biliary cirrhosis. Br J Dermatol 1978; 99: 447–50

    Article  PubMed  CAS  Google Scholar 

  25. Singhai PC, Scharschmidt LA. Membranous nephropathy associated with primary biliary cirrhosis and bullous pemphigoid. Ann Allergy 1985; 55: 484–5

    Google Scholar 

  26. Simon CA, Winkelmann RK. Bullous pemphigoid and glomeru-lonephritis. J Am Acad Dermatol 1986; 14: 456–63

    Article  PubMed  CAS  Google Scholar 

  27. Ross EA, Ahmed AR. Bullous pemphigoid-associated nephropathy: report of two cases and review of the literature. Am J Kidney Dis 1989; 19(3): 225–9

    Google Scholar 

  28. Bryning K, Leslie J. Factor V inhibitor and bullous pemphigoid. BMJ 1977; 1: 677–8

    Article  Google Scholar 

  29. Barth JH, Kelly SE, Wojnarowska F, et al. Pemphigoid and ulcerative colitis. J Am Acad Dermatol 1988; 19: 303–8

    Article  PubMed  CAS  Google Scholar 

  30. Harrison PV, Blewitt RW, Allen J, et al. Bullous pemphigoid and ulcerative colitis: a report of two cases and description of immunoblot findings. Br J Dermatol 1996; 134: 599–600

    Article  PubMed  CAS  Google Scholar 

  31. Nowicki MJ, Bishop PR, Parker PH. Bullous pemphigoid complicating Crohn’s disease in a child. Clin Pediatr 2002; 41: 59–62

    Article  Google Scholar 

  32. Chorzelski TP, Maciejowski E, Jablonska S, et al. Coexistence of pemphigus and bullous pemphigoid. Arch Dermatol 1974; 109: 849–53

    Article  PubMed  CAS  Google Scholar 

  33. Heine KG, Kumar A, Jordan RE. Pemphigus-like antibodies in bullous pemphigoid. Arch Dermatol 1977; 113: 1693–5

    Article  PubMed  CAS  Google Scholar 

  34. Leibovici V, Ron N, Goldenhersh M, et al. Coexistence of pemphigus and bullous pemphigoid. Int J Dermatol 1989; 28: 259–60

    Article  PubMed  CAS  Google Scholar 

  35. Ninomiya J, Nakabayashi A, Sei Y, et al. Bullous pemphigoid complicated with pemphigus vulgaris? Dermatology 1994; 189 Suppl. 1: 117–9

    Article  PubMed  Google Scholar 

  36. Sami N, Ahmed AR. Dual diagnosis of pemphigus and pemphigoid. Dermatology 2001; 202: 293–301

    Article  PubMed  CAS  Google Scholar 

  37. Sami N, Bhol KC, Beutner EH, et al. Diagnostic features of pemphigus vulgaris in patients with bullous pemphigoid. Dermatology 2002; 204: 108–17

    Article  PubMed  CAS  Google Scholar 

  38. Harrington CI, Sneddon IB. Coexistence of bullous pemphigoid and pemphigus foliaceus. Br J Dermatol 1979; 100: 441–5

    Article  PubMed  CAS  Google Scholar 

  39. Rantanen T, Niemi K. A further case of coexisting bullous pemphigoid and pemphigus foliaceus. Br J Dermatol 1979; 101: 611–2

    PubMed  CAS  Google Scholar 

  40. Korman NJ, Stanley JR, Woodley DT. Coexistence of pemphigus foliaceus and bullous pemphigoid. Arch Dermatol 1991; 127: 387–90

    Article  PubMed  CAS  Google Scholar 

  41. Bhat L, Sams HH, King LE. Bullous pemphigoid associated with Castleman’s disease. Arch Dermatol 2001; 137: 965–6

    PubMed  CAS  Google Scholar 

  42. Bachmeyer C, Seoud J, Carlotti A, et al. Bullous pemphigoid associated with acute myocarditis. Dermatology 2002; 204: 161–2

    Article  PubMed  Google Scholar 

  43. Ahmed AR, Kaplan RP, Hardy D, et al. Bullous pemphigoid and ulcerative colitis. Int J Dermatol 1982; 21: 594–8

    Article  PubMed  CAS  Google Scholar 

  44. Vaccaro M, D’Amico D, Borgia F, et al. Bullous pemphigoid following use of sulphasalazine for ulcerative colitis: drug-induced eruption or true association? Dermatology 2001; 203: 194–5

    Article  PubMed  CAS  Google Scholar 

  45. Barnadas MA, Gelpi C, Rocamora V, et al. Bullous pemphigoid associated with acute glomerulonephritis. Br J Dermatol 1998; 138: 867–71

    Article  PubMed  CAS  Google Scholar 

  46. Chosidow O, Doppler V, Bensimon G, et al. Bullous pemphigoid and amyotrophic lateral sclerosis: a new clue for understanding bullous pemphigoid? Arch Dermatol 2000; 136: 521–4

    Article  PubMed  CAS  Google Scholar 

  47. Grattan CEH. Evidence of an association between bullous pemphigoid and psoriasis. Br J Dermatol 1985; 113: 281–3

    Article  PubMed  CAS  Google Scholar 

  48. Stone SP, Schroeter AL. Bullous pemphigoid and associated malignant neoplasms. Arch Dermatol 1975; 111: 991–4

    Article  PubMed  CAS  Google Scholar 

  49. Ahmed AR, Amerian ML. Correlation of serum anti-basement membrane zone antibody and malignancy in bullous pemphigoid. Dermatologica 1985; 171: 82–5

    Article  PubMed  CAS  Google Scholar 

  50. Hadi SM, Barnetson RC, Gawkrodger DJ, et al. Clinical, histological and immunological studies in 50 patients with bullous pemphigoid. Dermatologica 1988; 176: 6–17

    Article  PubMed  CAS  Google Scholar 

  51. Lindelof B, Islam N, Eklund G, et al. Pemphigoid and cancer. Arch Dermatol 1990; 126: 66–8

    Article  PubMed  CAS  Google Scholar 

  52. Chang YT, Liu HN, Wong CK. Bullous pemphigoid: a report of 86 cases from Taiwan. Clin Exp Dermatol 1996; 21: 20–2

    Article  PubMed  CAS  Google Scholar 

  53. Hodge L, Marsden RA, Black MM, et al. Bullous pemphigoid: the frequency of mucosal involvement and concurrent malignancy related to indirect immunofluorescence findings. Br J Dermatol 1981; 105: 65–9

    Article  PubMed  CAS  Google Scholar 

  54. Binet O, Brunetiere RA, Rabary G, et al. Immunologic studies of bullous pemphigoid associated with adenocarcinoma of the colon. N Engl J Med 1983; 308(8): 460–1

    Article  PubMed  CAS  Google Scholar 

  55. Stanley JR, Hawley-Nelson P, Yuspa SH, et al. Characterization of bullous pemphigoid antigen: a unique basement membrane protein of stratified squamous epithelia. Cell 1981; 24: 897–903

    Article  PubMed  CAS  Google Scholar 

  56. Sawamura D, Li K, Chu ML, et al. Human bullous pemphigoid antigen (BPAG1). J Biol Chem 1991; 266(27): 17784–90

    PubMed  CAS  Google Scholar 

  57. Tanaka T, Parry DAD, Klaus-Kovtun V, et al. 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(19): 12555–9

    PubMed  CAS  Google Scholar 

  58. Borradori L, Sonnenberg A. Structure and function of hemidesmosomes: more than simple adhesion complexes. J Invest Dermatol 1999; 112: 411–8

    Article  PubMed  CAS  Google Scholar 

  59. Mutasim DF, Takahashi Y, Labib RS, et al. 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

    Article  PubMed  CAS  Google Scholar 

  60. Ishiko A, Shimizu H, Kikuchi A, et al. 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 hemis-desmosome in normal human and swine skin. J Clin Invest 1993; 91: 1608–15

    Article  PubMed  CAS  Google Scholar 

  61. Yang Y, Dowling J, Yu QC, et al. An essential cytoskeletal linker protein connecting actin microfilaments to intermediate filaments. Cell 1996; 86: 655–65

    Article  PubMed  CAS  Google Scholar 

  62. Borradori L, Chavanas S, Schaapveld RQJ, et al. Role of the bullous pemphigoid 180 (BP180) in the assembly of hemidesmosomes and cell adhesion: re-expression of BP180 in generalized atrophic benign epidermolysis bullosa keratinocytes. Exp Cell Res 1998; 239: 463–76

    Article  PubMed  CAS  Google Scholar 

  63. Schaapveld RQJ, Borradori L, Geerts D, et al. Hemidesmosomes formation is initiated by the β4 integrin subunit, requires complex formation with HD1/plectin and involves a direct interaction between β4 and the bullous pemphigoid antigen 180. J Cell Biol 1998; 142: 271–84

    Article  PubMed  CAS  Google Scholar 

  64. Okumura M, Yamakawa H, Ohara O, et al. Novel alternative splicings of BPAG1 (bullous pemphigoid antigen I) including the domain structure closely related to MACF (microtubule actin cross-linking factor). J Biol Chem 2002; 277(8): 6682–7

    Article  PubMed  CAS  Google Scholar 

  65. Mueller S, Klaus-Kovtun V, Stanley JR. A 230-kD basic protein is the major bullous pemphigoid antigen. J Invest Dermatol 1989; 92: 33–8

    Article  PubMed  CAS  Google Scholar 

  66. Hirako Y, Usukura J, Nishizawa N, et al. Demonstration of the molecular shape of BP180, a 180-kDa bullous pemphigoid antigen and its potential for trimer formation. J Biol Chem 1996; 271(23): 13739–45

    Article  PubMed  CAS  Google Scholar 

  67. Aho S, Uitto J. Direct interaction between the intracellular domains of bullous pemphigoid antigen 2 (BP180) and β4 integrin, hemidesmosomal components of basal keratinocytes. Biochem Biophys Res Commun 1998; 243: 694–8

    Article  PubMed  CAS  Google Scholar 

  68. Hopkinson SB, Findlay K, DeHart GW, et al. Interaction of BP180 (type XVII collagen) and α6 integrin is necessary for stabilization of hemidesmosome structure. J Invest Dermatol 1998; 111: 1015–22

    Article  PubMed  CAS  Google Scholar 

  69. Masunaga T, Shimizu H, Yee C, et al. The extracellular domain of BPAG2 localizes to anchoring filaments and its carboxyl terminus extends to the lamina densa of normal human epidermal basement membrane. J Invest Dermatol 1997; 109: 200–6

    Article  PubMed  CAS  Google Scholar 

  70. Giudice GJ, Squiquera HL, Elias PM, et al. Identification of two collagen domains within the bullous pemphigoid autoantigen, BP 180. J Clin Invest 1991; 87: 734–8

    Article  PubMed  CAS  Google Scholar 

  71. Giudice GJ, Emery DJ, Diaz LA. Cloning and primary structural analysis of the bullous pemphigoid autoantigen BP 180. J Invest Dermatol 1992; 99: 243–50

    Article  PubMed  CAS  Google Scholar 

  72. Li K, Giudice GJ, Tamai K, et al. Cloning of partial cDNA for mouse 180-kDa bullous pemphigoid antigen (BPAG2), a highly conserved collagenous protein of the cutaneous basement membrane zone. J Invest Dermatol 1992; 99: 258–63

    Article  PubMed  CAS  Google Scholar 

  73. Fairley JA, Heintz PW, Neuburg M, et al. Expression pattern of the bullous pemphigoid-180 antigen in normal and neoplastic epithelia. Br J Dermatol 1995; 133: 385–91

    Article  PubMed  CAS  Google Scholar 

  74. Liu Z, Diaz LA, Troy JL, et al. A passive transfer model of the organ-specific autoimmune disease, bullous pemphigoid, using antibodies generated against the hemidesmosomal antigen, BP 180. J Clin Invest 1993; 92: 2480–8

    Article  PubMed  CAS  Google Scholar 

  75. Hashimoto T, Ebihara T, Ishiko A, et al. Comparative study of bullous pemphigoid antigens among Japanese, British, and US patients indicates similar antigen profiles with the 170-kD antigen present both in the basement membrane and on the keratinocytes cell membrane. J Invest Dermatol 1993; 100: 385–9

    Article  PubMed  CAS  Google Scholar 

  76. Haase C, Budinger L, Borradori L, et al. 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: 282–6

    PubMed  CAS  Google Scholar 

  77. Korman NJ. In situ-bound antibodies eluted from the skin of patients with bullous pemphigoid are preferentially directed against the 230-kD bullous pemphigoid antigen. J Invest Dermatol 1995; 105: 324–830

    Article  Google Scholar 

  78. Ghohestani R, Kanitakis J, Nicolas JF, et al. Comparative sensitivity of indirect immunofluorescence to immunoblot assay for the detection of circulating antibodies to bullous pemphigoid antigens 1 and 2. Br J Dermatol 1996; 135: 74–9

    Article  PubMed  CAS  Google Scholar 

  79. Tanaka M, Hashimoto T, Dykes PJ, et al. Clinical manifestations in 100 Japanese bullous pemphigoid cases in relation to autoantigen profiles. Clin Exp Dermatol 1996; 21: 23–7

    Article  PubMed  CAS  Google Scholar 

  80. Jiao D, Bystryn JC. Relation between antibodies to BP180 and gender in bullous pemphigoid. J Am Acad Dermatol 1999; 41: 269–70

    Article  PubMed  CAS  Google Scholar 

  81. Bernard P, Bedane C, Bonnetblanc JM. Anti-BP180 autoantibodies as a marker of poor prognosis in bullous pemphigoid: a cohort analysis of 94 elderly patients. Br J Dermatol 1997; 136: 694–8

    Article  PubMed  CAS  Google Scholar 

  82. Liu Z, Diaz LA, Swartz SJ. Molecular mapping of a pathogenically relevant BP180 epitope associated with experimentally induced murine bullous pemphigoid. J Immunol 1995; 155: 5449–54

    PubMed  CAS  Google Scholar 

  83. Perriard J, Jaunin F, Favre B, et al. IgG autoantibodies from bullous pemphigoid (BP) patients bind antigenic sites on both the extracellular and intracellular domains of the BP antigen 180. J Invest Dermatol 1999; 112: 141–7

    Article  PubMed  CAS  Google Scholar 

  84. Giudice GJ, Emery DG, Zelickson BD, et al. Bullous pemphigoid and herpes gestationis autoantibodies recognize a common non-collagenous site on the BP180 ectodomain. J Immunol 1993; 151: 5742–50

    PubMed  CAS  Google Scholar 

  85. Matsumura K, Amagai M, Nishikawa T, et al. The majority of bullous pemphigoid and herpes gestationis serum samples react with the N16a domain of the 180-kDa bullous pemphigoid antigen. Arch Dermatol Res 1996; 288: 507–9

    Article  PubMed  CAS  Google Scholar 

  86. Zillikens D, Rose PA, Balding SD, et al. Tight clustering of extracellular BP180 epitopes recognized by bullous pemphigoid autoantibodies. J Invest Dermatol 1997; 109: 573–9

    Article  PubMed  CAS  Google Scholar 

  87. Schmidt E, Obe K, Brocker DB, et al. Serum levels of autoantibodies to BP180 correlate with disease activity in patients with bullous pemphigoid. Arch Dermatol 2000; 136: 174–8

    Article  PubMed  CAS  Google Scholar 

  88. Sitaru C, Schmidt E, Petermann S, et al. Autoantibodies to bullous pemphigoid antigen 180 induce dermal-epidermal separation in cryosections of human skin. J Invest Dermatol 2002; 118: 664–71

    Article  PubMed  CAS  Google Scholar 

  89. Zillikens D, Giudice GJ. BP180/type XVII collagen: its role in acquired and inherited disorders or the dermal-epidermal junction. Arch Dermatol Res 1999; 291: 187–94

    Article  PubMed  CAS  Google Scholar 

  90. Bird P, Friedmann PS, Ling N, et al. Subclass distribution of IgG autoantibodies in bullous pemphigoid. J Invest Dermatol 1986; 86: 21–5

    Article  PubMed  CAS  Google Scholar 

  91. Laffitte E, Skaria M, Jaunin F, et al. 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: 760–8

    Article  PubMed  CAS  Google Scholar 

  92. Wintroub BU, Mihm MC, Goetzl EJ, et al. Morphologic and functional evidence for release of mast-cell products in bullous pemphigoid. N Engl J Med 1978; 298: 417–21

    Article  PubMed  CAS  Google Scholar 

  93. Dvorak AM, Mihm MC, Osage JE, et al. Bullous pemphigoid, an ultrastructural study of the inflammatory response: eosinophil, basophil and mast granule changes in multiple biopsies from one patient. J Invest Dermatol 1982; 78: 91–101

    Article  PubMed  CAS  Google Scholar 

  94. Chan LS, Vanderlugt CJ, Hashimoto T, et al. Epitope spreading: Lessons from autoimmune skin diseases. J Invest Dermatol 1998; 110: 103–9

    Article  PubMed  CAS  Google Scholar 

  95. Skaria M, Jaunin F, Hunziker T, et al. IgG autoantibodies from bullous pemphigoid patients recognise multiple antigenic reactive sites located predominantly within the B and C subdomain of the COOH-terminus of BP 230. J Invest Dermatol 2000; 114: 1–7

    Article  Google Scholar 

  96. Stanley JR, Tanaka T, Mueller S, et al. Isolation of complementary DNA for bullous pemphigoid antigen by use of patients’ autoantibodies. J Clin Invest 1988; 82: 1864–70

    Article  PubMed  CAS  Google Scholar 

  97. Rico MJ, Korman NJ, Stanley JR, et al. IgG antibodies from patients with bullous pemphigoid bind to localized epitopes on synthetic peptides encoded by bullous peniphigoid antigen cDNA. J Immunol 1990; 145: 3728–33

    PubMed  CAS  Google Scholar 

  98. Tanaka M, Hashimoto T, Amagai M, et al. Characterization of bullous pemphigoid antibodies by use of recombinant bullous pemphigoid antigen proteins. J Invest Dermatol 1991; 97: 725–8

    Article  PubMed  CAS  Google Scholar 

  99. Gaucherand M, Nicolas JF, Paranhos Baccala G, et al. Major antigenic epitopes of bullous pemphigoid 230 kDa antigen map within the C-terminal end of the protein: evidence using a 55 kDa recombinant protein. Br J Dermatol 1995; 132: 190–6

    Article  PubMed  CAS  Google Scholar 

  100. Ghohestani RF, Cozzani E, Delaporte E, et al. IgE antibodies in sera from patients with bullous pemphigoid are autoantibodies preferentially directed against the 230-kDa epidermal antigen (BP230). J Clin Immunol 1998; 18(3): 202–9

    Article  PubMed  CAS  Google Scholar 

  101. Dopp E, Schmidt E, Chimanovitch I, et al. 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: 577–83

    PubMed  CAS  Google Scholar 

  102. Bernard P, Aucouturier P, Denis F, et al. Immunoblot analysis of IgG subclasses of circulating antibodies in bullous pemphigoid. Clin Immunol Immunopathol 1990; 54: 484–94

    Article  PubMed  CAS  Google Scholar 

  103. Parodi A, Rebora A. Serum IgE antibodies bind to the epidermal side of the basement membrane zone splits in bullous pemphigoid. Br J Dermatol 1992; 126: 526–7

    Article  PubMed  CAS  Google Scholar 

  104. Soh H, Hosokawa H, Asada Y. IgE and its related phenomena in bullous pemphigoid. Br J Dermatol 1993; 128: 371–7

    Article  PubMed  CAS  Google Scholar 

  105. Schmidt E, Brocker E, Zillikens D. High levels of soluble CD23 in blister fluid of patients with bullous pemphigoid. Arch Dermatol 1995; 131: 966–7

    Article  PubMed  CAS  Google Scholar 

  106. Delaporte E, Dubost-Brama A, Ghohestani R, et al. IgE autoantibodies directed against the major bullous pemphigoid antigen in patients with a severe form of pemphigoid. J Immunol 1996; 157: 3642–7

    PubMed  CAS  Google Scholar 

  107. Labib RS, Anhalt GJ, Patel HP, et al. Molecular heterogeneity of the bullous pemphigoid antigens as detected by immunoblotting. J Immunol 1986; 136(4): 1231–5

    PubMed  CAS  Google Scholar 

  108. Kirtschig G, Wojnarowska F. IgA basement membrane zone autoantibodies in bullous pemphigoid detect epidermal antigens of 270–280 kDa, 230 kDa, and 180 kDa molecular weight by immunoblotting. Clin Exp Dermatol 1999; 24: 302–7

    Article  PubMed  CAS  Google Scholar 

  109. Schacke H, Schumann H, Hammami-Hauasli N, et al. Two forms of collagen XVII in keratinocytes. J Biol Chem 1998; 273(40): 25937–43

    Article  PubMed  CAS  Google Scholar 

  110. Schaller J, Giese T, Ladusch M, et al. Interleukin-2 receptor expression and interleukin-2 production in bullous pemphigoid. Arch Dermatol Res 1990; 282: 223–6

    Article  PubMed  CAS  Google Scholar 

  111. Dubucquoi S, Desreumaux P, Janin A, et al. Interleukin-5 synthesis by eosinophils: association with granules and immunoglobulin-dependent secretion. J Exp Med 1994; 179: 703–8

    Article  PubMed  CAS  Google Scholar 

  112. Ameglio F, D’Auria L, Bonifati C, et al. Cytokine pattern in blister fluid and serum of patients with bullous pemphigoid: relationships with disease intensity. Br J Dermatol 1998; 138: 611–4

    Article  PubMed  CAS  Google Scholar 

  113. Schmidt E, Bastian B, Dummer R, et al. Detection of elevated levels of IL-r, IL-6, and IL-10 in blister fluid of bullous pemphigoid. Arch Dermatol Res 1996; 288: 353–7

    Article  PubMed  CAS  Google Scholar 

  114. Xu L, Robinson N, Miller SD, et al. Characterization of BALB/c mice B lymphocyte autoimmune responses to skin basement membrane component type XVII collagen, the target antigen of autoimmune skin disease bullous pemphigoid. Immunol Lett 2001; 77: 105–11

    Article  PubMed  CAS  Google Scholar 

  115. Jordon RE, Kawana S, Fritz KA. Immunopathologic mechanisms in pemphigus and pemphigoid. J Invest Dermatol 1985; 85 (1 Suppl.): 72S–8S

    Article  PubMed  CAS  Google Scholar 

  116. Liu Z, Giudice GJ, Swartz SJ, et al. The role of complement in experimental bullous pemphigoid. J Clin Invest 1995; 95: 1539–44

    Article  PubMed  CAS  Google Scholar 

  117. Liu Z, Giudice GJ, Zhou X, et al. A major role for neutrophils in experimental Bullous pemphigoid. J Clin Invest 1995; 100: 1256–63

    Article  Google Scholar 

  118. Chen R, Ning G, Zhao M, et al. Mast cells play a key role in neutrophils recruitment in experimental bullous pemphigoid. J Clin Invest 2001; 108(8): 1151–8

    PubMed  CAS  Google Scholar 

  119. Gammon WR, Merritt CC, Lewis DM, et al. An in vitro model of immune complex-mediated basement membrane zone separation caused by pemphigoid antibodies, leukocytes and complement. J Invest Dermatol 1982; 78: 285–90

    Article  PubMed  CAS  Google Scholar 

  120. Liu Z, Shipley JM, Vu TH, et al. Gelatinase B-deficient mice are resistant to experimental Bullous pemphigoid. J Exp Med 1998; 188(3): 475–82

    Article  PubMed  CAS  Google Scholar 

  121. Stahle-Backdahl M, Inoue M, Giudice G, et al. 92-kD gelatinase is produced by eosinophils at the site of blister formation in Bullous pemphigoid and cleaves the extracellular domain of recombinant 180-dD Bullous pemphigoid autoantigen. J Clin Invest 1994; 93: 2022–30

    Article  PubMed  CAS  Google Scholar 

  122. Verraes S, Hornebeck W, Polette M, et al. 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: 1091–6

    Article  PubMed  CAS  Google Scholar 

  123. Weiss SJ. Tissue destruction by neutrophils. N Engl J Med 1989; 320: 265–376

    Article  Google Scholar 

  124. Yancey KB. From bedside to bench and back: the diagnosis and biology of bullous diseases. Arch Dermatol 1994; 130: 983–7

    Article  PubMed  CAS  Google Scholar 

  125. Nousari HC, Anhalt GJ. Pemphigus and bullous pemphigoid. Lancet 1999; 354: 667–72

    Article  PubMed  CAS  Google Scholar 

  126. Ghohestani RF, Novotney J, Chaudhary M, et al. Bullous pemphigoid: From the bedside to the research laboratory. Clin Dermatol 2001; 19: 690–6

    Article  PubMed  CAS  Google Scholar 

  127. Goldberg DJ, Sabolinski M, Bystryn JC. Regional variation in the expression of bullous pemphigoid antigen and location of lesions in bullous pemphigoid. J Invest Dermatol 1984; 82: 326–8

    Article  PubMed  CAS  Google Scholar 

  128. Asbrink E, Hovmark A. Clinical variations in bullous pemphigoid with respect to early symptoms. Acta Dermatovener (Stockholm) 1981; 61: 417–21

    CAS  Google Scholar 

  129. Amato DA, Silverstein J, Zitelli J. The prodrome of bullous pemphigoid. Int J Dermatol 1988; 27: 560–3

    Article  PubMed  CAS  Google Scholar 

  130. Wolf R, Ophir J, Dechner E. Nonbullous bullous pemphigoid. Int J Dermatol 1992; 31: 498–500

    Article  PubMed  CAS  Google Scholar 

  131. Strohal R, Rappersberger K, Pehamberger H, et al. Nonbullous pemphigoid: prodrome of bullous pemphigoid or a distinct pemphigoid variant? J Am Acad Dermatol 1993; 29: 293–9

    Article  PubMed  CAS  Google Scholar 

  132. Alonso-Llamazares J, Rogers RS, Oursler JR, et al. Bullous pemphigoid presenting as generalized pruritus: observations in six patients. Int J Dermatol 1998; 37: 508–14

    PubMed  CAS  Google Scholar 

  133. Liu HN, Su WPD, Rogers RS. Clinical variants of pemphigoid. Int J Dermatol 1986; 25: 17–27

    Article  PubMed  CAS  Google Scholar 

  134. Powell AM, Albert S, Gratian MJ, et al. Pemphigoid nodularis (non-bullous): a clinicopathological study of five cases. Br J Dermatol 2002; 147: 343–9

    Article  PubMed  CAS  Google Scholar 

  135. Person JR, Rogers RS, Perry HO. Localized pemphigoid. Br J Dermatol 1976; 95: 531–4

    Article  PubMed  CAS  Google Scholar 

  136. Provost TT, Maize JC, Ahmed AR, et al. Unusual subepidermal bullous diseases with immunologic features of bullous pemphigoid. Arch Dermatol 1979; 115: 156–60

    Article  PubMed  CAS  Google Scholar 

  137. Borradori L, Prost C, Wolkenstein P, et al. Localized pretibial pemphigoid and pemphigoid nodularis. J Am Acad Dermatol 1992; 27: 863–7

    Article  PubMed  CAS  Google Scholar 

  138. Ernst TM, Marsch WCH. Development of localized pemphigoid following radiation treatment. Dermatolgica 1982; 164: 73–81

    Article  CAS  Google Scholar 

  139. Parslew R, Verbov JL. Bullous pemphigoid at sites of trauma. Br J Dermatol 1997; 137: 825–6

    Article  PubMed  CAS  Google Scholar 

  140. Ghura HS, Johnston GA, Milligan A. Development of a bullous pemphigoid after split-skin grafting. Br J Plast Surg 2001; 54: 447–9

    Article  PubMed  CAS  Google Scholar 

  141. Anderson CK, Mowad CM, Goff ME, et al. Bullous pemphigoid arising in surgical wounds. Br J Dermatol 2001; 145: 670–2

    Article  PubMed  CAS  Google Scholar 

  142. Yesudian PD, Dobson CM, Ahmad R, et al. Trauma-induced bullous pemphigoid around venous access site in a hemodialysis patient. Clin Exp Dermatol 2002; 27: 70–2

    Article  PubMed  CAS  Google Scholar 

  143. Levine N, Freilich A, Barland P. Localized pemphigoid simulating dyshidrosiform dermatitis. Arch Dermatol 1979; 115: 320–1

    Article  PubMed  CAS  Google Scholar 

  144. Massa MC, Connolly SM. Bullous pemphigoid with features of prurigo nodularis. Arch Dermatol 1982; 118: 937–9

    Article  PubMed  CAS  Google Scholar 

  145. Borradori L, Rybojad M, Verola O, et al. Pemphigoid nodularis. Arch Dermatol 1990; 126: 1522–3

    Article  PubMed  CAS  Google Scholar 

  146. Ross JS, McKee PH, Smith NP, et al. Unusual variants of pemphigoid: from pruritus to pemphigoid nodularis. J Cutan Pathol 1992; 19: 212–6

    Article  PubMed  CAS  Google Scholar 

  147. Gallo R, Parodi A, Rebora A. Pemphigoid nodularis. Br J Dermatol 1993; 129: 744–5

    Article  PubMed  CAS  Google Scholar 

  148. Bourke JF, Berth-Jones J, Gawkrodger DJ, et al. Pemphigoid nodularis: a report of two cases. Clin Exp Dermatol 1994; 19: 496–9

    Article  PubMed  CAS  Google Scholar 

  149. Schmidt E, Sitaru C, Schubert B, et al. Subacute prurigo variant of bullous pemphigoid: autoantibodies show the same specificity compared with classic bullous pemphigoid. J Am Acad Dermatol 2002; 47: 133–6

    Article  PubMed  Google Scholar 

  150. Schachter M, Brieva JC, Jones JCR, et al. Pemphigoid nodularis associated with autoantibodies to the NC16A domain of BP180 and a hyperproliferative integrin profile. J Am Acad Dermatol 2001; 45: 747–54

    Article  PubMed  CAS  Google Scholar 

  151. Tani M, Murata Y, Masaki H. Pemphigoid nodularis. J Am Acad Dermatol 1989; 21: 1099–104

    Article  PubMed  CAS  Google Scholar 

  152. Cliff S, Holden CA. Pemphigoid nodularis: a report of three cases and review of the literature. Br J Dermatol 1997; 136: 398–401

    Article  PubMed  CAS  Google Scholar 

  153. Yung SW, Soltani K, Lorincz AL. Pemphigoid nodularis. J Am Acad Dermatol 1981; 5: 54–60

    Article  PubMed  CAS  Google Scholar 

  154. Gengoux P, Lachapelle JM. Pemphigoid presenting as atypical excoriated prurigo: regarding 11 cases. Dermatol 1997; 194: 392–4

    Article  CAS  Google Scholar 

  155. Winkelmann RK, Su WPD. Pemphigoid vegetans. Arch Dermatol 1979; 115: 446–8

    Article  PubMed  CAS  Google Scholar 

  156. Kuokkanen K, Helin H. Pemphigoid vegetans. Arch Dermatol 1981; 117: 56–7

    Article  PubMed  CAS  Google Scholar 

  157. Chan LS, Dorman MA, Agha A, et al. Pemphigoid vegetans represents a bullous pemphigoid variant. J Am Acad Dermatol 1993; 28: 331–5

    Article  PubMed  CAS  Google Scholar 

  158. Korman NJ, Woods SG. Erythrodermic bullous pemphigoid is a clinical variant of bullous pemphigoid. Br J Dermatol 1995; 133: 967–71

    Article  PubMed  CAS  Google Scholar 

  159. Amato L, Gallerani I, Mei S, et al. Erythrodermic bullous pemphigoid. Int J Dermatol 2001; 40: 343–8

    Article  PubMed  CAS  Google Scholar 

  160. Tappeiner G, Konrad K, Holubar K. Erythrodermic bullous pemphigoid. J Am Acad Dermatol 1982; 6: 489–92

    Article  PubMed  CAS  Google Scholar 

  161. Saitoh A, Atsushi O, Ohtake N. Erythrodermic bullous pemphigoid. J Am Acad Dermatol 1993; 28: 124–5

    Article  PubMed  CAS  Google Scholar 

  162. Bean SF, Michel B, Furey N, et al. Vesicular pemphigoid. Arch Dermatol 1976; 112: 1402–4

    Article  PubMed  CAS  Google Scholar 

  163. Jawitz J, Kumar V, Nigra TP, et al. Vesicular pemphigoid vs dermatitis herpetiformis. J Am Acad Dermatol 1984; 10: 892–6

    Article  PubMed  CAS  Google Scholar 

  164. Sander HM, Utz MMP, Peters MS. Bullous pemphigoid and dermatitis herpetiformis: mixed bullous disease or coexistence of two separate entities? J Cutan Pathol 1989; 16: 370–4

    Article  PubMed  CAS  Google Scholar 

  165. Honeyman JF, Honeyman AR, De la Parra MA, et al. Polymorphic pemphigoid. Arch Dermatol 1979; 115: 423–7

    Article  PubMed  CAS  Google Scholar 

  166. Cordel N, Courville P, Martel P, et al. Extensive erosive bullous pemphigoid: an atypical serious clinical variant. Br J Dermatol 2002; 146: 537–9

    Article  PubMed  CAS  Google Scholar 

  167. Lang PG, Maize JC. Coexisting lichen planus and bullous pemphigoid or lichen planus pemphigoides? J Am Acad Der-matol 1983; 9: 133–40

    Article  Google Scholar 

  168. Mora RG, Nesbitt LT, Brantley JB. Lichen planus pemphigoides: clinical and immunofluorescent findings in four cases. J Am Acad Dermatol 1983; 8: 331–6

    Article  PubMed  CAS  Google Scholar 

  169. Davis AL, Bhogal BS, Whitehead P, et al. Lichen planus pemphigoides: its relationship to bullous pemphigoid. Br J Dermatol 1991; 125: 263–71

    Article  PubMed  CAS  Google Scholar 

  170. Zillikens D, Caux F, Mascaro JM, et al. Autoantibodies in lichen planus pemphigoides react with a novel epitope within the c-terminal NC16A domain of BP 180. J Invest Dermatol 1999; 113: 117–21

    Article  PubMed  CAS  Google Scholar 

  171. Stingl G, Holubar K. Coexistence of lichen planus and bullous pemphigoid: an immunopathological study. Br J Dermatol 1975; 93: 313–20

    Article  PubMed  CAS  Google Scholar 

  172. Joly P, Tanasescu S, Wolkenstein P, et al. Lichenoid erythrodermic bullous pemphigoid of the African patient. J Am Acad Dermatol 1998; 39: 691–7

    Article  PubMed  CAS  Google Scholar 

  173. Nemeth AJ, Klein AD, Gould EW, et al. Childhood bullous pemphigoid. Arch Dermatol 1991; 127: 378–86

    Article  PubMed  CAS  Google Scholar 

  174. Bean SF, Good RA, Windhorst DB. Bullous pemphigoid in an 11 year old boy. Arch Dermatol 1970; 102: 205–8

    Article  PubMed  CAS  Google Scholar 

  175. Fincher DF, Dupree E, Bean SF. Bullous pemphigoid in childhood. Arch Dermatol 1971; 103: 88–90

    Article  PubMed  CAS  Google Scholar 

  176. Ostlere LS, Stevens H, Black MM, et al. Bullous pemphigoid in infancy: a case report including new immunoblotting observations. Clin Exp Dermatol 1993; 18: 483–5

    Article  PubMed  CAS  Google Scholar 

  177. Ratnavel RC, Shanks AJ, Grant JW. Juvenile pemphigoid nodularis. Br J Dermatol 1994; 130: 125–35

    Article  PubMed  CAS  Google Scholar 

  178. Ruocco V, Sacerdoti G. Pemphigus and bullous pemphigoid due to drugs. Int J Dermatol 1991; 30: 307–12

    Article  PubMed  CAS  Google Scholar 

  179. Fellner MJ. Drug-induced bullous pemphigoid. Clin Dermatol 1993; 11: 515–20

    Article  PubMed  CAS  Google Scholar 

  180. Czechowicz RT, Reid CM, Warren LJ, et al. Bullous pemphigoid induced by cephalexin. Australas J Dermatol 2001; 42: 152–5

    Google Scholar 

  181. Bart B, Bean S. Bullous pemphigoid following the topical use of fluorouracil. Arch Dermatol 1970; 102: 457–60

    Article  PubMed  CAS  Google Scholar 

  182. Thomsen K, Schmidt H. PUVA-induced bullous pemphigoid. Br J Dermatol 1976; 95: 568–9

    Article  PubMed  CAS  Google Scholar 

  183. Duschet P, Schwarz S, Gschnait F. Bullous pemphigoid after radiation therapy. J Am Acad Dermatol 1988; 18: 441–4

    Article  PubMed  CAS  Google Scholar 

  184. Fournier B, Descamps V, Bouscarat F, et al. Bullous pemphigoid induced by vaccination. Br J Dermatol 1996; 135: 144–61

    Article  Google Scholar 

  185. Lear JT, Tan BB, English JSC. Bullous pemphigoid following influenza vaccination [letter]. Clin Exp Dermatol 1996; 21(5): 392

    Article  PubMed  CAS  Google Scholar 

  186. Millard TP, Smith HR, Black MM, et al. Bullous pemphigoid developing during systemic therapy with chloroquine. Clin Exp Dermatol 1999; 24: 263–5

    Article  PubMed  CAS  Google Scholar 

  187. Alcalay J, David M, Ingber A, et al. Bullous pemphigoid mimicking bullous erythema multiforme: an untoward side effect of penicillins. J Am Acad Dermatol 1988; 18: 345–9

    Article  PubMed  CAS  Google Scholar 

  188. Hodak E, Ben-Shetrit A, Ingber A, et al. Bullous pemphigoid: an adverse effect of ampicillin. Clin Exp Dermatol 1990; 15: 50–2

    Article  PubMed  CAS  Google Scholar 

  189. Delbado C, Rieckhoff-Cantoni L, Helg C, et al. Bullous pemphigoid associated with acute graft-versus-host disease after allogeneic bone marrow transplantation. Bone Marrow Transplant 1992; 10: 377–9

    Google Scholar 

  190. Miralles J, Barnadas MA, Baselga E, et al. Bullous pemphigoid-like lesions induced by amoxicillin. Int J Dermatol 1997; 36: 42–7

    Article  PubMed  CAS  Google Scholar 

  191. Velthuis PJ, Hendrikse JC, Nefkens JJ. Combined features of pemphigus and pemphigoid induced by penicillamine. Br J Dermatol 1985; 112: 615–9

    Article  PubMed  CAS  Google Scholar 

  192. Rasmussen HB, Jepsen LV, Brandrup F. Penicillamine-induced bullous pemphigoid with pemphigus-like antibodies. J Cutan Pathol 1989; 16: 154–7

    Article  PubMed  CAS  Google Scholar 

  193. Fellner MJ, Katz JM. Occurrence of bullous pemphigoid after furosemide therapy. Arch Dermatol 1976; 112: 75–7

    Article  PubMed  CAS  Google Scholar 

  194. Koch CA, Mazzaferri EL, Larry JA, et al. Bullous pemphigoid after treatment with furosemide. Cutis 1996; 58: 340–4

    PubMed  CAS  Google Scholar 

  195. Kashihara M, Danno K, Miyachi Y, et al. Bullous pemphigoid-like lesions induced by phenacetin. Arch Dermatol 1984; 120: 1196–9

    Article  PubMed  CAS  Google Scholar 

  196. Pazderka Smith E, Taylor TB, Meyer LJ, et al. Antigen identification in drug-induced bullous pemphigoid. J Am Acad Dermatol 1993; 29: 879–82

    Article  Google Scholar 

  197. Laing VB, Sherertz EF, Flowers FP. Pemphigoid-like bullous eruption related to ibuprofen. J Am Acad Dermatol 1988; 19: 91–4

    Article  PubMed  CAS  Google Scholar 

  198. Downs AMR, Lear JT, Bower CPR, et al. Does influenza vaccination induce bullous pemphigoid? A report of four cases [letter]. Br J Dermatol 1998; 138(2): 363

    Article  PubMed  CAS  Google Scholar 

  199. Rault S, Grosieux-Dauger C, Verrae S, et al. Bullous pemphigoid induced by fluoxetine. Br J Dermatol 1999; 141: 755–6

    Article  PubMed  CAS  Google Scholar 

  200. Grange F, Scrivener Y, Koessler A, et al. Spironolactone-induced pemphigoid. Ann Dermatol Venereol 1997; 124: 700–2

    PubMed  CAS  Google Scholar 

  201. Boulinguez S, Bernard P, Bédane C, et al. Bullous pemphigoid induced by bumetanide. Br J Dermatol 1998; 138: 548–9

    Article  PubMed  CAS  Google Scholar 

  202. Bastuji-Garin S, Joly P, Picard-Dahan C, et al. Drugs associated with bullous pemphigoid. Arch Dermatol 1996; 132: 272–6

    Article  PubMed  CAS  Google Scholar 

  203. Morrison LH, Labib RS, Zone JJ, et al. Herpes gestationis autoantibodies recognize a 180-kD human epidermal antigen. J Clin Invest 1988; 81: 2023–6

    Article  PubMed  CAS  Google Scholar 

  204. Gee BC, Allen J, Khumalo NP, et al. Bullous pemphigoid in pregnancy: contrasting behaviour in two patients. Br J Dermatol 2001; 145: 994–7

    Article  PubMed  CAS  Google Scholar 

  205. Bedane C, McMillan JR, Balding SD, et al. Bullous pemphigoid and cicatricial pemphigoid autoantibodies react with ultras-tructurally separable epitopes on the BP180 ectodomain: evidence that BP180 spans the lamina lucida. J Invest Dermatol 1997; 108: 901–7

    Article  PubMed  CAS  Google Scholar 

  206. Gammon WR, Kowalewski C, Chorzelski TP, et al. Direct immunofluorescence studies of sodium chloride-separated skin in the differential diagnosis of bullous pemphigoid and epidermolysis bullosa acquisita. J Am Acad Dermatol 1990; 22: 664–70

    Article  PubMed  CAS  Google Scholar 

  207. Gammon WR, Briggaman RA, Wheeler CE. Epidermolysis bullosa acquisita presenting as an inflammatory bullous disease. J Am Acad Dermatol 1982; 7: 382–7

    Article  PubMed  CAS  Google Scholar 

  208. Gammon WR, Briggaman RA, Woodley DT, et al. Epidermolysis bullosa acquisita: a pemphigoid-like disease. J Am Acad Dermatol 1984; 11: 820–32

    Article  PubMed  CAS  Google Scholar 

  209. Tindall JG, Rea TH, Shulman I, et al. Herpes gestationis in association with a hydatidiform mole. Arch Dermatol 1981; 117: 510–2

    Article  PubMed  CAS  Google Scholar 

  210. Shornick JK, Bangert JL, Freeman RJ, et al. Herpes gestationis: clinical and histologic features of twenty-eight cases. J Am Acad Dermatol 1983; 8: 214–24

    Article  PubMed  CAS  Google Scholar 

  211. Lin M, Arteaga LA, Diaz LA. Herpes gestationis. Clin Dermatol 2001; 19: 697–702

    Article  PubMed  CAS  Google Scholar 

  212. Jenkins RE, Vaughan Jones SA, Black MM. Conversion of pemphigoid gestationis to bullous pemphigoid: two refractory cases highlighting this association. Br J Dermatol 1995; 135: 595–8

    Article  Google Scholar 

  213. Vaillant L, Bernard P, Joly P, et al. Evaluation of clinical criteria for diagnosis of bullous pemphigoid. Arch Dermatol 1998; 134: 1075–80

    Article  PubMed  CAS  Google Scholar 

  214. Bernard P, Venot J, Constant F, et al. Blood eosinophilia as a severity marker for bullous pemphigoid. J Am Acad Dermatol 1987; 16: 879–81

    Article  PubMed  CAS  Google Scholar 

  215. Bushkell LL, Jordon RE. Bullous pemphigoid: a cause of peripheral blood eosinophilia. J Am Acad Dermatol 1983; 8: 648–51

    Article  PubMed  CAS  Google Scholar 

  216. Arbesman CE, Wypych JI, Reisman RE, et al. IgE levels in sera of patients with pemphigus or bullous pemphigoid. Arch Dermatol 1974; 110: 378–81

    Article  PubMed  CAS  Google Scholar 

  217. Heilborn JD, Stahle-Backdahl M, Albertioni F, et al. Low-dose oral pulse methotrexate monotherapy in elderly patients with bullous pemphigoid. J Am Acad Dermatol 1999; 40: 741–9

    Article  PubMed  CAS  Google Scholar 

  218. Schaumburg-Lever G, Orfanos CE, Lever WF. Electron microscopic study of bullous pemphigoid. Arch Dermatol 1972; 106: 662–7

    Article  PubMed  CAS  Google Scholar 

  219. Nishioka K, Hashimoto K, Katayama I, et al. Eosinophilic spongiosis in bullous pemphigoid. Arch Dermatol 1984; 120: 1166–8

    Article  PubMed  CAS  Google Scholar 

  220. Kirtschig G, Wojnarowska F. Autoimmune blistering diseases: an up-date of diagnostic methods and investigations. Clin Exp Dermatol 1994; 19: 97–112

    Article  PubMed  CAS  Google Scholar 

  221. Weigand DA. Effect of anatomic region on immunofluorescence diagnosis of bullous pemphigoid. J Am Acad Dermatol 1985; 12: 274–8

    Article  PubMed  CAS  Google Scholar 

  222. Gammon WR, Fine J, Forbes M, et al. Immunofluorescence on split skin for the detection and differentiation of basement membrane zone autoantibodies. J Am Acad Dermatol 1992; 27: 79–87

    Article  PubMed  CAS  Google Scholar 

  223. Kelly SE, Wojnarowska F. The use of chemically split tissue in the detection of circulating anti-basement membrane zone antibodies in bullous pemphigoid and cicatricial pemphigoid. Br J Dermatol 1988; 118: 31–40

    Article  PubMed  CAS  Google Scholar 

  224. Hachisuka H, Kurose K, Karashima T, et al. Serum from normal elderly individuals contains antibasement membrane zone antibodies. Arch Dermatol 1996; 132: 1201–5

    Article  PubMed  CAS  Google Scholar 

  225. Logan RA, Bhogal B, Das AK, et al. Localization of bullous pemphigoid antibody: an indirect immunofluorescence study of 228 cases using a split-skin technique. Br J Dermatol 1987; 117: 471–8

    Article  PubMed  CAS  Google Scholar 

  226. Ghohestani RF, Nicolas JF, Rousselle P, et al. Diagnostic value of indirect immunofluorescence on sodium chloride-split skin in differential diagnosis of subepidermal autoimmune bullous dermatoses. Arch Dermatol 1997; 133: 1102–7

    Article  PubMed  CAS  Google Scholar 

  227. Batteux F, Franck N, Jaffray P, et al. An extract from cultured human keratinocytes that contains the major autoantigens related to autoimmune bullous skin diseases. J Clin Immunol 1997; 17(3): 228–33

    Article  PubMed  CAS  Google Scholar 

  228. Bernard P, Didierjean L, Denis F, et al. Heterogeneous bullous pemphigoid antibodies: detection and characterization by immunoblotting when absent by indirect immunofluorescence. J Invest Dermatol 1989; 92: 171–4

    Article  PubMed  CAS  Google Scholar 

  229. Machado P, Michalaki H, Roche P, et al. Serological diagnosis of bullous pemphigoid (BP): comparison of the sensitivity of indirect immunofluorescence on salt-split skin to immunoblotting. Br J Dermatol 1992; 126: 236–41

    Article  PubMed  CAS  Google Scholar 

  230. Rieckhoff-Cantoni L, Bernard P, Didierjean L, et al. Frequency of bullous pemphigoid-like antibodies as detected by Western immunoblot analysis in pruritic dermatoses. Arch Dermatol 1992; 128: 791–4

    Article  PubMed  CAS  Google Scholar 

  231. Zillikens D, Mascaro JM, Rose PA, et al. A highly sensitive enzyme-linked immunosorbant assay for the detection of cir-culating anti-BP180 autoantibodies in patients with bullous pemphigoid. J Invest Dermatol 1997; 109: 679–83

    Article  PubMed  CAS  Google Scholar 

  232. Wojnarowska F, Kirtschig G, Khumalo N. Treatment of subepidermal immunobullous diseases. Clin Dermatol 2001; 19: 768–77

    Article  PubMed  CAS  Google Scholar 

  233. Wojnarowska F, Kirtschig G, Highet AS, et al. Guidelines for the management of bullous pemphigoid. Br J Dermatol 2002; 147: 214–21

    Article  PubMed  CAS  Google Scholar 

  234. Ahmed AR. Intravenous immunoglobulin therapy for patients with bullous pemphigoid unresponsive to conventional immu-nosuppressive treatment. J Am Acad Dermatol 2001; 45: 825–35

    Article  PubMed  CAS  Google Scholar 

  235. Westerhof W. Treatment of bullous pemphigoid with topical clobetasol propionate. J Am Acad Dermatol 1989; 20: 458–61

    Article  PubMed  CAS  Google Scholar 

  236. Paquet PH, Richelle M, Lapiere CM. Bullous pemphigoid treated by topical corticosteroids. Acta Derm Venereol (Stockh) 1991; 71: 534–5

    CAS  Google Scholar 

  237. Muramatsu T, Lida T, Shira T. Pemphigoid and pemphigus foliaceus successfully treated with topical corticosteroids. J Dermatol 1996; 23: 683–8

    PubMed  CAS  Google Scholar 

  238. Claudy A. Evaluation of the safety and efficacy of a potent topical cortico-steroid in the treatment of bullous pemphigoid. Clin Dermatol 2001; 19: 778–80

    Article  PubMed  CAS  Google Scholar 

  239. Bystryn JC, Wainwright BD, Shupack JL. Oral and topical steroids in bullous pemphigoid. N Engl J Med 2002; 347(2): 143–5

    Article  PubMed  Google Scholar 

  240. Joly P, Roujeau JC, Benichou J, et al. A comparison of oral and topical corticosteroids in patients with bullous pemphigoid. N Engl J Med 2002; 346(5): 321–7

    Article  PubMed  CAS  Google Scholar 

  241. Ardern-Jones MR, Venning VA, Wojnarowska F. Oral and topical corticosteroids in bullous pemphigoid. N Engl J Med 2002; 347(2): 143–5

    Article  PubMed  Google Scholar 

  242. Stern RS. Bullous pemphigoid therapy: think globally, act locally. N Engl J Med 2002; 346(5): 364–7

    Article  PubMed  Google Scholar 

  243. Korman NJ. Oral and topical corticosteroids in bullous pemphigoid. N Engl J Med 2002; 347(2): 143–5

    Article  PubMed  Google Scholar 

  244. Spigel GT. Oral and topical corticosteroids in bullous pemphigoid [letter]. N Engl J Med 2002; 347(2): 143–5

    Article  PubMed  Google Scholar 

  245. Dreno B, Sassolas B, Lacour P, et al. Methylprednisolone versus prednisolone methylsulfobenzoate in pemphigoid: a comparative multicenter study. Ann Dermatol Venereol 1993; 120: 518–21

    PubMed  CAS  Google Scholar 

  246. Khumalo NP, Murrell DF, Wojnarowska F, et al. A systemic review of treatments for bullous pemphigoid. Arch Dermatol 2002; 138: 385–9

    Article  PubMed  Google Scholar 

  247. Lebrun-Vignes JC, Roujeau R, Bernard P, et al. Prednisone is more effective than prednisolone metasulfobenzoate in the treatment of bullous pemphigoid. Arch Dermatol 1999; 135: 89–90

    Article  PubMed  CAS  Google Scholar 

  248. Siegel J, Eaglstein WH. High-dose methylprednisolone in the treatment of bullous pemphigoid. Arch Dermatol 1984; 120: 1157–65

    Article  PubMed  CAS  Google Scholar 

  249. Mutasim DF. Treatment considerations while awaiting the ideal bullous pemphigoid trial. Arch Dermatol 2002; 138(3): 404

    Article  PubMed  Google Scholar 

  250. Morel P, Guillaume JC. Treatment of bullous pemphigoid with prednisolone only: 0.75 mg/kg/day versus 1.25 mg/kg/day: a multicenter randomized study. Ann Dermatol Venereol 1984; 111: 925–8

    PubMed  CAS  Google Scholar 

  251. Fine JD. Management of acquired bullous skin diseases. N Engl J Med 1995; 333(22): 1475–84

    Article  PubMed  CAS  Google Scholar 

  252. Thornfeldt CR, Menkes AW. Bullous pemphigoid controlled by tetracycline. J Am Acad Dermatol 1987; 16: 305–10

    Article  PubMed  CAS  Google Scholar 

  253. Berk MA, Lorincz AL. The treatment of bullous pemphigoid with tetracycline and niacinamide. Arch Dermatol 1986; 122: 670–4

    Article  PubMed  CAS  Google Scholar 

  254. Kolbach DN, Remme JJ, Bos WH, et al. Bullous pemphigoid successfully controlled by tetracycline and nicotinamide. Br J Dermatol 1995; 133: 88–90

    Article  PubMed  CAS  Google Scholar 

  255. Fox BJ, Odom RB, Findlay RF. Erythromycin therapy in bullous pemphigoid: possible anti-inflammatory effects. J Am Acad Dermatol 1982; 7: 504–10

    Article  PubMed  CAS  Google Scholar 

  256. Loo WJ, Kirtschig G, Wojnarowska F. Minocycline as a therapeutic option in bullous pemphigoid. Clin Exp Dermatol 2001: 26: 376–9

    Article  PubMed  CAS  Google Scholar 

  257. Thomas I, Khorenian S, Arbesfeld DM. Treatment of generalized bullous pemphigoid with oral tetracycline. J Am Acad Dermatol 1993; 28: 74–7

    Article  PubMed  CAS  Google Scholar 

  258. Hornschuh B, Hamm H, Wever S, et al. Treatment of 16 patients with bullous pemphigoid with oral tetracycline and niacinamide and topical clobetasol. J Am Acad Dermatol 1997; 36: 101–3

    Article  PubMed  CAS  Google Scholar 

  259. Fivenson DP, Breneman DL, Rosen GB, et al. Nicotinamide and tetracycline therapy of bullous pemphigoid. Arch Dermatol 1994; 130: 753–8

    Article  PubMed  CAS  Google Scholar 

  260. Chaidemenos GC. Tetracycline and niacinamide in the treatment of blistering skin diseases. Clin Dermatol 2001; 19: 781–5

    Article  PubMed  CAS  Google Scholar 

  261. Bekier E, Wyczolkowska J, Szych H, et al. The inhibitory effect of nicotinamide on asthma-like symptoms and eosinophilia in guinea pigs, anaphylactic mast cell degranulation in mice, and histamine release from rat peritoneal mast cells by compound 48/ 80. Int Arch Allergy Appl Immunol 1974; 47: 737–48

    Article  PubMed  CAS  Google Scholar 

  262. Esterly NB, Furey NL, Flanagan LE. The effect of antimicrobial agents on leukocyte chemotaxis. J Invest Dermatol 1978; 70: 51–5

    Article  PubMed  CAS  Google Scholar 

  263. Humbert P, Treffel P, Chapius JF, et al. The tetracyclines in dermatology. J Am Acad Dermatol 1991; 25: 691–7

    Article  PubMed  CAS  Google Scholar 

  264. Sanchez Miralles E, Nunez Cabezon M, Ledo Pozueta A. Treatment of generalized bullous pemphigoid with oral tetracycline [letter]. J Am Acad Dermatol 1994; 30 (2 Pt 1): 291

    Article  Google Scholar 

  265. Burton JL, Harman RRM, Peachey RDG, et al. Azathioprine plus prednisone in treatment of pemphigoid. BMJ 1978; 2: 1190–1

    Article  PubMed  CAS  Google Scholar 

  266. Burton JL, Greaves MW. Azathioprine for pemphigus and pemphigoid: a 4 year follow-up. Br J Dermatol 1974; 91: 103–9

    Article  PubMed  CAS  Google Scholar 

  267. Guillaume JC, Vaillant L, Bernard P, et al. Controlled trial of azathioprine and plasma exchange in addition to prednisolone in the treatment of bullous pemphigoid. Arch Dermatol 1993: 129: 49–53

    Article  PubMed  CAS  Google Scholar 

  268. Anstey A. Controlled trial of azathioprine and plasma exchange in addition to prednisolone in the treatment of bullous pemphigoid. Arch Dermatol 1993; 129: 1203–4

    Article  PubMed  CAS  Google Scholar 

  269. Paul MA, Jorizzo JL, Fleischer AB, et al. Low-dose methotrex-ate treatment in elderly patients with bullous pemphigoid. J Am Acad Dermatol 1994; 31: 620–5

    Article  PubMed  CAS  Google Scholar 

  270. Olsen EA. The pharmacology of methotrexate. J Am Acad Dermatol 1991; 25: 306–18

    Article  PubMed  CAS  Google Scholar 

  271. Bohm M, Beissert S, Schwarz T, et al. Bullous pemphigoid treated with mycophenolate mofetil [letter]. Lancet 1997; 349: 541

    Article  PubMed  CAS  Google Scholar 

  272. Nousari HC, Griffin WA, Anhalt GJ. Successful therapy for bullous pemphigoid with mycophenolate mofetil. J Am Acad Dermatol 1998; 39: 497–8

    Article  PubMed  CAS  Google Scholar 

  273. Grundmann-Kollmann M, Korting HC, Behrens S, et al. Mycophenolate mofetil: a new therapeutic option in the treatment of blistering autoimmune diseases. J Am Acad Dermatol 1999; 40: 957–60

    Article  PubMed  CAS  Google Scholar 

  274. Person JR, Rogers RS. Bullous pemphigoid responding to sulphapyridine and the sulphones. Arch Dermatol 1977; 113: 610–5

    Article  PubMed  CAS  Google Scholar 

  275. Piamphongsant T. Dapsone for the treatment of bullous pemphigoid. Asian Pac J Allergy Immunol 1983; 1: 19–21

    PubMed  CAS  Google Scholar 

  276. Venning VA, Millard PR, Wojnarowska F. Dapsone as first line therapy for bullous pemphigoid. Br J Dermatol 1989; 120: 83–92

    Article  PubMed  CAS  Google Scholar 

  277. Stendahl O, Molin L, Dahlgren C. The inhibition of polymorphonuclear cytotoxicity by dapsone. J Clin Invest 1978; 62: 214–20

    Article  PubMed  CAS  Google Scholar 

  278. Milligan A, Hutchinson PE. The use of chlorambucil in the treatment of bullous pemphigoid. J Am Acad Dermatol 1990; 22: 796–801

    Article  PubMed  CAS  Google Scholar 

  279. Itoh T, Hosokawa H, Shirai Y, et al. Successful treatment of bullous pemphigoid with pulsed intravenous cyclophos-phamide. Br J Dermatol 1996; 134: 931–3

    Article  PubMed  CAS  Google Scholar 

  280. Dawe RS, Naidoo DK, Ferguson J. Severe bullous pemphigoid responsive to pulsed intravenous dexamethasone and oral cyclophosphamide. Br J Dermatol 1997; 137: 826–7

    Article  PubMed  CAS  Google Scholar 

  281. Thivolet J, Barthelemy H, Rigot-Muller G, et al. Effects of cyclosporin on bullous pemphigoid and pemphigus. Lancet 1985; I: 334–5

    Article  Google Scholar 

  282. Barthelemy H, Thivolet J, Cambazard F, et al. Cyclosporin in the treatment of bullous pemphigoid: preliminary study. Ann Dermatol Vernereol 1986; 113: 309–13

    CAS  Google Scholar 

  283. Cunliffe WJ. Bullous pemphigoid and response to cyclosporin. Br J Dermatol 1987; 32: 113–4

    Article  Google Scholar 

  284. Nousari HC, Anhalt GJ. Bullous pemphigoid treated with leflunomide. Arch Dermatol 2000; 136: 1204–5

    Article  PubMed  CAS  Google Scholar 

  285. Guillot B, Donadio D, Guilhou JJ, et al. Plasma exchanges in the treatment of bullous pemphigoid. Presse Med 1983; 12(30): 1855–8

    PubMed  CAS  Google Scholar 

  286. Egan CA, Meadows KP, Zone JJ. Plasmapheresis as a steroid saving procedure in bullous pemphigoid. Int J Dermatol 2000; 39: 230–5

    Article  PubMed  CAS  Google Scholar 

  287. Wollina U, Lange D, Looks A. Short-time extracorporeal photochemotherapy in the treatment of drug-resistant autoimmune bullous diseases. Dermatology 1999; 198: 140–4

    Article  PubMed  CAS  Google Scholar 

  288. Roujeau JC, Morel P, Dalle E, et al. Plasma exchange in bullous pemphigoid. Lancet 1984; II: 486–8

    Article  Google Scholar 

  289. Guillot B, Donadio D, Guilhou JJ, et al. Long term plasma exchange therapy in bullous pemphigoid. Acta Derm Venereol (Stockh) 1985; 66: 73–5

    Google Scholar 

  290. Beckers RCY, Brand A, Vermeer BJ, et al. Adjuvant high-dose intravenous gammaglobulin in the treatment of pemphigus and bullous pemphigoid: experience in six patients. Br J Dermatol 1995; 133: 289–93

    Article  PubMed  CAS  Google Scholar 

  291. Tappeiner G, Steiner A. High-dosage intravenous gamma globulin: therapeutic failure in pemphigus and pemphigoid. J Am Acad Dermatol 1989; 20: 684–5

    Article  PubMed  CAS  Google Scholar 

  292. Harman KE, Black MM. High-dose intravenous immune globulin for the treatment of autoimmune blistering diseases: an evaluation of its use in 14 cases. Br J Dermatol 1999; 140: 865–74

    Article  PubMed  CAS  Google Scholar 

  293. Engineer L, Ahmed AR. Role of intravenous immunoglobulin in the treatment of bullous pemphigoid: analysis of current data. J Am Acad Dermatol 2000; 44: 83–8

    Article  Google Scholar 

  294. Rutter A, Luger TA. High-dose intravenous immunoglobulins: an approach to treat severe immune-mediated and autoimmune diseases of the skin. J Am Acad Dermatol 2001; 44: 1010-24

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgements

The authors have no conflicts of interest that are directly relevant to the content of this review. They would like to thank the Canadian Institute for Health Research (PRM) and the Canadian Dermatology Foundation (PRM) for their generous support of this work.

Author information

Authors and Affiliations

  1. Division of Dermatology, University of Toronto, Toronto, Ontario, Canada

    Scott R. A. Walsh

  2. Department of Medicine, University of Toronto, Toronto, Ontario, Canada

    David Hogg

  3. Division of Dermatology, Departments of Medicine and Medical Genetics, University of Calgary, 3330 Hospital Dr NW, Calgary, Alberta, Canada, T2N 4N1

    P. Régine Mydlarski

Authors
  1. Scott R. A. Walsh
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. David Hogg
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. P. Régine Mydlarski
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to P. Régine Mydlarski.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Walsh, S.R.A., Hogg, D. & Régine Mydlarski, P. Bullous Pemphigoid. CNS Drugs 65, 905–926 (2005). https://doi.org/10.2165/00003495-200565070-00002

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.2165/00003495-200565070-00002

Keywords

Search

Navigation