Pemphigoid diseases

The Lancet

Volumn 381, Issue 9863, 2013, Pages 320-332

  Schmidt, Enno ^a,b   Zillikens, Detlef ^a  

^a UNIVERSITY OF LÜBECK   (Germany)

^b University of Luabeck   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

AZATHIOPRINE; BP180 PROTEIN; BULLOUS PEMPHIGOID ANTIGEN 1; CETIRIZINE; CHLORAMBUCIL; COLCHICINE; CORTICOSTEROID; CYCLOPHOSPHAMIDE; DAPSONE; IMMUNOGLOBULIN; MEMBRANE PROTEIN; METHOTREXATE; MYCOPHENOLIC ACID; PREDNISOLONE; SULFAPYRIDINE; TETRACYCLINE; UNCLASSIFIED DRUG;

ARTICLE; BULLOUS PEMPHIGOID; CORTICOSTEROID THERAPY; DISEASE ASSOCIATION; ENZYME LINKED IMMUNOSORBENT ASSAY; EPIDERMOLYSIS BULLOSA ACQUISITA; HISTOPATHOLOGY; HUMAN; IMMUNOFLUORESCENCE MICROSCOPY; INCIDENCE; LICHEN PLANUS; LINEAR IGA BULLOUS DERMATOSIS; MOLECULAR PATHOLOGY; MORTALITY; MUCOUS MEMBRANE PEMPHIGOID; NONHUMAN; PEMPHIGOID; PEMPHIGOID GESTATIONIS; PRIORITY JOURNAL; RISK FACTOR; SEROLOGY;

EID: 84872937506     PISSN: 01406736     EISSN: 1474547X     Source Type: Journal    
DOI: 10.1016/S0140-6736(12)61140-4     Document Type: Review

References (150)

1. Ghohestani RF, Hudson BG, Claudy A, Uitto J. The α5 chain of type IV collagen is the target of IgG autoantibodies in a novel autoimmune disease with subepidermal blisters and renal insuffi ciency. J Biol Chem 2000; 275: 16002-06.
2. Chan LS, Fine JD, Briggaman RA, et al. Identifi cation and partial characterization of a novel 105-kDalton lower lamina lucida autoantigen associated with a novel immune-mediated subepidermal blistering disease. J Invest Dermatol 1993; 101: 262-67.
3. Venning VA. Linear IgA disease: Clinical presentation, diagnosis, and pathogenesis. Dermatol Clin 2011; 29: 453-58.
4. Lever WF. Pemphigus. Medicine (Baltimore) 1953; 32: 1-123.
5. Jordon RE, Beutner EH, Witebsky E, Blumental G, Hale WL, Lever WF. Basement zone antibodies in bullous pemphigoid. JAMA 1967; 200: 751-56.
6. Marazza G, Pham HC, Scharer L, et al. Incidence of bullous pemphigoid and pemphigus in Switzerland: A 2-year prospective study. Br J Dermatol 2009; 161: 861-68.
7. Bertram F, Brocker EB, Zillikens D, Schmidt E. Prospective analysis of the incidence of autoimmune bullous disorders in Lower Franconia, Germany. J Dtsch Dermatol Ges 2009; 7: 434-40.
8. Gudi VS, White MI, Cruickshank N, et al. Annual incidence and mortality of bullous pemphigoid in the Grampian region of north-east Scotland. Br J Dermatol 2005; 153: 424-27.
9. Joly P, Baricault S, Sparsa A, et al. Incidence and mortality of bullous pemphigoid in France. J Invest Dermatol 2012; 132: 1998-2004.
10. Langan SM, Smeeth L, Hubbard R, Fleming KM, Smith CJ, West J. Bullous pemphigoid and pemphigus vulgaris-incidence and mortality in the UK: Population based cohort study. BMJ 2008; 337: 160-63.
11. Jung M, Kippes W, Messer G, Zillikens D, Rzany B. Increased risk of bullous pemphigoid in male and very old patients: A population-based study on incidence. J Am Acad Dermatol 1999; 41: 266-68.
12. Waisbourd-Zinman O, Ben-Amitai D, Cohen AD, et al. Bullous pemphigoid in infancy: Clinical and epidemiologic characteristics. J Am Acad Dermatol 2008; 58: 41-48.
13. Cortes B, Marazza G, Naldi L, Combescure C, Borradori L. Mortality of bullous pemphigoid in Switzerland: A prospective study. Br J Dermatol 2011; 165: 368-74.
14. 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: 321-27.
15. Rzany B, Partscht K, Jung M, et al. Risk factors for lethal outcome in patients with bullous pemphigoid: Low serum albumin level, high dosage of glucocorticosteroids, and old age. Arch Dermatol 2002; 138: 903-08.
16. Taghipour K, Chi CC, Vincent A, Groves RW, Venning V, Wojnarowska F. The association of bullous pemphigoid with cerebrovascular disease and dementia: A case-control study. Arch Dermatol 2010; 146: 1251-54.
17. Chen YJ, Wu CY, Lin MW, et al. Comorbidity profiles among patients with bullous pemphigoid: A nationwide population-based study. Br J Dermatol 2011; 165: 593-99.
18. Langan SM, Groves RW, West J. The relationship between neurological disease and bullous pemphigoid: A population-based case-control study. J Invest Dermatol 2010; 131: 631-36.
19. Bastuji-Garin S, Joly P, Lemordant P, et al. Risk factors for bullous pemphigoid in the elderly: A prospective case-control study. J Invest Dermatol 2011; 131: 637-43.
20. Seppanen A, Miettinen R, Alafuzoff I. Neuronal collagen XVII is localized to lipofuscin granules. Neuroreport 2010; 21: 1090-94.
21. Claudepierre T, Manglapus MK, Marengi N, et al. Collagen XVII and BPAG1 expression in the retina: Evidence for an anchoring complex in the central nervous system. J Comp Neurol 2005; 487: 190-203.
22. Guo L, Degenstein L, Dowling J, et al. Gene targeting of BPAG1: Abnormalities in mechanical strength and cell migration in stratifi ed epithelia and neurologic degeneration. Cell 1995; 81: 233-43.
23. Taylor G, Venning V, Wojnarowska F, Welch K. Bullous pemphigoid and autoimmunity. J Am Acad Dermatol 1993; 29: 181-84.
24. Lindelof B, Islam N, Eklund G, Arfors L. Pemphigoid and cancer. Arch Dermatol 1990; 126: 66-68.
25. Ogawa H, Sakuma M, Morioka S, et al. The incidence of internal malignancies in pemphigus and bullous pemphigoid in Japan. J Dermatol Sci 1995; 9: 136-41.
26. Venning VA, Wojnarowska F. Induced bullous pemphigoid. Br J Dermatol 1995; 132: 831-32.
27. Walmsley N, Hampton P. Bullous pemphigoid triggered by swine fl u vaccination: Case report and review of vaccine triggered pemphigoid. J Dermatol Case Rep 2011; 5: 74-76.
28. Bastuji-Garin S, Joly P, Picard-Dahan C, et al. Drugs associated with bullous pemphigoid-a case-control study. Arch Dermatol 1996; 132: 272-76.
29. Schmidt E, della Torre R, Borradori L. Clinical features and practical diagnosis of bullous pemphigoid. Dermatol Clin 2011; 29: 427-38.
30. Murrell DF, Daniel BS, Joly P, et al. Defi nitions and outcome measures for bullous pemphigoid: Recommendations by an international panel of experts. J Am Acad Dermatol 2011; 66: 479-85.
31. Diaz LA, Ratrie H 3rd, Saunders WS, et al. 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: 1088-94.
32. 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: 1231-35.
33. Stanley JR, Hawley-Nelson P, Yuspa SH, Shevach EM, Katz SI. Characterization of bullous pemphigoid antigen: A unique basement membrane protein of stratifi ed squamous epithelia. Cell 1981; 24: 897-903.
34. 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: 5742-50.
35. Perriard J, Jaunin F, Favre B, et al. IgG autoantibodies from bullous pemphigoid (BP) patients bind antigenic sites on both the extracellular and the intracellular domains of the BP antigen 180. J Invest Dermatol 1999; 112: 141-47.
36. Hofmann S, Thoma-Uszynski S, Hunziker T, et al. Severity and phenotype of bullous pemphigoid relate to autoantibody profile against the NH2-and COOH-terminal regions of the BP180 ectodomain. J Invest Dermatol 2002; 119: 1065-73.
37. Di Zenzo G, Thoma-Uszynski S, Fontao L, et al. Multicenter prospective study of the humoral autoimmune response in bullous pemphigoid. Clin Immunol 2008; 128: 415-26.
38. Di Zenzo G, Grosso F, Terracina M, et al. Characterization of the anti-BP180 autoantibody reactivity profile and epitope mapping in bullous pemphigoid patients. J Invest Dermatol 2004; 122: 103-10.
39. Kromminga A, Scheckenbach C, Georgi M, et al. Patients with bullous pemphigoid and linear IgA disease show a dual IgA and IgG autoimmune response to BP180. J Autoimmun 2000; 15: 293-300.
40. 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 refl ect disease activity. J Am Acad Dermatol 2000; 42: 577-83.
41. Iwata Y, Komura K, Kodera M, et al. Correlation of IgE autoantibody to BP180 with a severe form of bullous pemphigoid. Arch Dermatol 2008; 144: 41-48.
42. Christophoridis S, Budinger L, Borradori L, Hunziker T, Merk HF, Hertl M. IgG, IgA and IgE autoantibodies against the ectodomain of BP180 in patients with bullous and cicatricial pemphigoid and linear IgA bullous dermatosis. Br J Dermatol 2000; 143: 349-55.
43. Skaria M, Jaunin F, Hunziker T, et al. IgG autoantibodies from bullous pemphigoid patients recognize multiple antigenic reactive sites located predominantly within the B and C subdomains of the COOH-terminus of BP230. J Invest Dermatol 2000; 114: 998-1004.
44. 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: 3642-47.
45. Di Zenzo G, Thoma-Uszynski S, Calabresi V, et al. Demonstration of epitope-spreading phenomena in bullous pemphigoid: Results of a prospective multicenter study. J Invest Dermatol 2011; 131: 2271-80.
46. Kobayashi M, Amagai M, Kuroda-Kinoshita K, et al. BP180 ELISA using bacterial recombinant NC16a protein as a diagnostic and monitoring tool for bullous pemphigoid. J Dermatol Sci 2002; 30: 224-32.
47. Schmidt E, Obe K, Brocker EB, Zillikens D. Serum levels of autoantibodies to BP180 correlate with disease activity in patients with bullous pemphigoid. Arch Dermatol 2000; 136: 174-78.
48. Iwata H, Kamio N, Aoyama Y, et al. IgG from patients with bullous pemphigoid depletes cultured keratinocytes of the 180-kDa bullous pemphigoid antigen (type XVII collagen) and weakens cell attachment. J Invest Dermatol 2009; 129: 919-26.
49. Schmidt E, Reimer S, Kruse N, et al. Autoantibodies to BP180 associated with bullous pemphigoid release interleukin-6 and interleukin-8 from cultured human keratinocytes. J Invest Dermatol 2000; 115: 842-48.
50. Messingham KN, Srikantha R, DeGueme AM, Fairley JA. FcR-independent eff ects of IgE and IgG autoantibodies in bullous pemphigoid. J Immunol 2011; 187: 553-60.
51. Gammon WR, Merritt CC, Lewis DM, Sams WM Jr, Carlo JR, Wheeler CE Jr. 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.
52. 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: 664-71.
53. Bieber K, Sun S, Ishii N, et al. Animal models for autoimmune bullous dermatoses. Exp Dermatol 2009; 19: 2-11.
54. Liu Z, Diaz LA, Troy JL, et al. A passive transfer model of the organ-specifi c autoimmune disease, bullous pemphigoid, using antibodies generated against the hemidesmosomal antigen, BP180. J Clin Invest 1993; 92: 2480-88.
55. Lin L, Bankaitis E, Heimbach L, et al. Dual targets for mouse mast cell protease-4 in mediating tissue damage in experimental bullous pemphigoid. J Biol Chem 2011; 286: 37358-67.
56. Olasz EB, Roh J, Yee CL, et al. Human bullous pemphigoid antigen 2 transgenic skin elicits specifi c IgG in wild-type mice. J Invest Dermatol 2007; 127: 2807-17.
57. Nishie W, Sawamura D, Goto M, et al. Humanization of autoantigen. Nat Med 2007; 13: 378-83.
58. Liu Z, Sui W, Zhao M, et al. Subepidermal blistering induced by human autoantibodies to BP180 requires innate immune players in a humanized bullous pemphigoid mouse model. J Autoimmun 2008; 31: 331-38.
59. Hirose M, Recke A, Beckmann T, et al. Repetitive immunization breaks tolerance to type XVII collagen and leads to bullous pemphigoid in mice. J Immunol 2011; 187: 1176-83.
60. Ujiie H, Shibaki A, Nishie W, et al. A novel active mouse model for bullous pemphigoid targeting humanized pathogenic antigen. J Immunol 2010; 184: 2166-74.
61. Ujiie H, Shibaki A, Nishie W, et al. Noncollagenous 16A domain of type XVII collagen-reactive CD4+ T cells play a pivotal role in the development of active disease in experimental bullous pemphigoid model. Clin Immunol 2012; 142: 167-75.
62. Budinger L, Borradori L, Yee C, et al. Identifi cation and characterization of autoreactive T cell responses to bullous pemphigoid antigen 2 in patients and healthy controls. J Clin Invest 1998; 102: 2082-89.
63. Thoma-Uszynski S, Uter W, Schwietzke S, Schuler G, Borradori L, Hertl M. Autoreactive T and B cells from bullous pemphigoid (BP) patients recognize epitopes clustered in distinct regions of BP180 and BP230. J Immunol 2006; 176: 2015-23.
64. Kasperkiewicz M, Zillikens D, Schmidt E. Pemphigoid diseases: Pathogenesis, diagnosis, and treatment. Autoimmunity 2012; 45: 55-70.
65. Fairley JA, Burnett CT, Fu CL, Larson DL, Fleming MG, Giudice GJ. A pathogenic role for IgE in autoimmunity: Bullous pemphigoid IgE reproduces the early phase of lesion development in human skin grafted to nu/nu mice. J Invest Dermatol 2007; 127: 2605-11.
66. Zone JJ, Taylor T, Hull C, Schmidt L, Meyer L. IgE basement membrane zone antibodies induce eosinophil infi ltration and histological blisters in engrafted human skin on SCID mice. J Invest Dermatol 2007; 127: 1167-74.
67. Fairley JA, Baum CL, Brandt DS, Messingham KA. Pathogenicity of IgE in autoimmunity: Successful treatment of bullous pemphigoid with omalizumab. J Allergy Clin Immunol 2009; 123: 704-05.
68. Hall RP 3rd, Murray JC, McCord MM, Rico MJ, Streilein RD. Rabbits immunized with a peptide encoded for by the 230-kD bullous pemphigoid antigen cDNA develop an enhanced infl ammatory response to UVB irradiation: A potential animal model for bullous pemphigoid. J Invest Dermatol 1993; 101: 9-14.
69. Kiss M, Husz S, Janossy T, et al. Experimental bullous pemphigoid generated in mice with an antigenic epitope of the human hemidesmosomal protein BP230. J Autoimmun 2005; 24: 1-10.
70. Yoshida M, Hamada T, Amagai M, et al. Enzyme-linked immunosorbent assay using bacterial recombinant proteins of human BP230 as a diagnostic tool for bullous pemphigoid. J Dermatol Sci 2006; 41: 21-30.
71. Gammon WR, Briggaman RA, Inman AO 3rd, Queen LL, Wheeler CE. Diff erentiating anti-lamina lucida and anti-sublamina densa anti-BMZ antibodies by indirect immunofl uorescence on 1?0 M sodium chloride-separated skin. J Invest Dermatol 1984; 82: 139-44.
72. Sitaru C, Dahnrich C, Probst C, et al. Enzyme-linked immunosorbent assay using multimers of the 16th non-collagenous domain of the BP180 antigen for sensitive and specifi c detection of pemphigoid autoantibodies. Exp Dermatol 2007; 16: 770-77.
73. Blocker IM, Dahnrich C, Probst C, et al. Epitope mapping of BP230 leading to a novel enzyme-linked immunosorbent assay for autoantibodies in bullous pemphigoid. Br J Dermatol 2012; 166: 964-70.
74. Charneux J, Lorin J, Vitry F, et al. Usefulness of BP230 and BP180-NC16a enzyme-linked immunosorbent assays in the initial diagnosis of bullous pemphigoid: A retrospective study of 138 patients. Arch Dermatol 2011; 147: 286-91.
75. Roussel A, Benichou J, Randriamanantany ZA, et al. Enzyme-linked immunosorbent assay for the combination of bullous pemphigoid antigens 1 and 2 in the diagnosis of bullous pemphigoid. Arch Dermatol 2011; 147: 293-98.
76. Bernard P, Reguiai Z, Tancrede-Bohin E, et al. Risk factors for relapse in patients with bullous pemphigoid in clinical remission: A multicenter, prospective, cohort study. Arch Dermatol 2009; 145: 537-42.
77. Hofmann SC, Tamm K, Hertl M, Borradori L. Diagnostic value of an enzyme-linked immunosorbent assay using BP180 recombinant proteins in elderly patients with pruritic skin disorders. Br J Dermatol 2003; 149: 910-12.
78. Wieland CN, Comfere NI, Gibson LE, Weaver AL, Krause PK, Murray JA. Anti-bullous pemphigoid 180 and 230 antibodies in a sample of unaff ected subjects. Arch Dermatol 2010; 146: 21-25.
79. Rose C, Schmidt E, Kerstan A, et al. Histopathology of anti-laminin 5 mucous membrane pemphigoid. J Am Acad Dermatol 2009; 61: 433-40.
80. Rose C, Weyers W, Denisjuk N, Hillen U, Zillikens D, Shimanovich I. Histopathology of anti-p200 pemphigoid. Am J Dermatopathol 2007; 29: 119-24.
81. Kirtschig G, Middleton P, Bennett C, Murrell DF, Wojnarowska F, Khumalo NP. Interventions for bullous pemphigoid. Cochrane Database Syst Rev 2010; 10: CD002292.
82. Joly P, Roujeau JC, Benichou J, et al. A comparison of two regimens of topical corticosteroids in the treatment of patients with bullous pemphigoid: A multicenter randomized study. J Invest Dermatol 2009; 129: 1681-87.
83. Kjellman P, Eriksson H, Berg P. A retrospective analysis of patients with bullous pemphigoid treated with methotrexate. Arch Dermatol 2008; 144: 612-16.
84. Schmidt E, Kraensel R, Goebeler M, et al. Treatment of bullous pemphigoid with dapsone, methylprednisolone, and topical clobetasol propionate: A retrospective study of 62 cases. Cutis 2005; 76: 205-09.
85. Chave TA, Mortimer NJ, Shah DS, Hutchinson PE. Chlorambucil as a steroid-sparing agent in bullous pemphigoid. Br J Dermatol 2004; 151: 1107-08.
86. Du-Thanh A, Merlet S, Maillard H, et al. Combined treatment with low-dose methotrexate and initial short-term superpotent topical steroids in bullous pemphigoid: An open, multicentre, retrospective study. Br J Dermatol 2011; 165: 1337-43.
87. Chan LS, Ahmed AR, Anhalt GJ, et al. The first international consensus on mucous membrane pemphigoid: Defi nition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol 2002; 138: 370-79.
88. Bernard P, Vaillant L, Labeille B, et al. Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Bullous Diseases French Study Group. Arch Dermatol 1995; 131: 48-52.
89. Thorne JE, Anhalt GJ, Jabs DA. Mucous membrane pemphigoid and pseudopemphigoid. Ophthalmology 2004; 111: 45-52.
90. Ahmed AR, Kurgis BS, Rogers RS 3rd. Cicatricial pemphigoid. J Am Acad Dermatol 1991; 24: 987-1001.
91. Delgado JC, Turbay D, Yunis EJ, et al. A common major histocompatibility complex class II allele HLA-DQB1 0301 is present in clinical variants of pemphigoid. Proc Natl Acad Sci USA 1996; 93: 8569-71.
92. Saw VP, Dart JK. Ocular mucous membrane pemphigoid: Diagnosis and management strategies. Ocul Surf 2008; 6: 128-42.
93. Egan CA, Lazarova Z, Darling TN, Yee C, Cote T, Yancey KB. Anti-epiligrin cicatricial pemphigoid and relative risk for cancer. Lancet 2001; 357: 1850-51.
94. Oyama N, Setterfield JF, Powell AM, et al. Bullous pemphigoid antigen II (BP180) and its soluble extracellular domains are major autoantigens in mucous membrane pemphigoid: The pathogenic relevance to HLA class II alleles and disease severity. Br J Dermatol 2006; 154: 90-98.
95. Schmidt E, Skrobek C, Kromminga A, et al. Cicatricial pemphigoid: IgA and IgG autoantibodies target epitopes on both intra-and extracellular domains of bullous pemphigoid antigen 180. Br J Dermatol 2001; 145: 778-83.
96. Domloge-Hultsch N, Gammon WR, Briggaman RA, Gil SG, Carter WG, Yancey KB. Epiligrin, the major human keratinocyte integrin ligand, is a target in both an acquired autoimmune and an inherited subepidermal blistering skin disease. J Clin Invest 1992; 90: 1628-33.
97. Balding SD, Prost C, Diaz LA, et al. Cicatricial pemphigoid autoantibodies react with multiple sites on the BP180 extracellular domain. J Invest Dermatol 1996; 106: 141-46.
98. Kirtschig G, Marinkovich MP, Burgeson RE, Yancey KB. Anti-basement membrane autoantibodies in patients with anti-epiligrin cicatricial pemphigoid bind the alpha subunit of laminin 5. J Invest Dermatol 1995; 105: 543-48.
99. Rashid KA, Gurcan HM, Ahmed AR. Antigen specifi city in subsets of mucous membrane pemphigoid. J Invest Dermatol 2006; 126: 2631-36.
100. Lazarova Z, Yee C, Darling T, Briggaman RA, Yancey KB. Passive transfer of anti-laminin 5 antibodies induces subepidermal blisters in neonatal mice. J Clin Invest 1996; 98: 1509-18.
101. Lazarova Z, Hsu R, Yee C, Yancey KB. Human anti-laminin 5 autoantibodies induce subepidermal blisters in an experimental human skin graft model. J Invest Dermatol 2000; 114: 178-84.
102. Lazarova Z, Hsu R, Briggaman RA, Yancey KB. Fab fragments directed against laminin 5 induce subepidermal blisters in neonatal mice. Clin Immunol 2000; 95: 26-32.
103. Rashid KA, Stern JN, Ahmed AR. Identifi cation of an epitope within human integrin α6 subunit for the binding of autoantibody and its role in basement membrane separation in oral pemphigoid. J Immunol 2006; 176: 1968-77.
104. Chan RY, Bhol K, Tesavibul N, et al. The role of antibody to human β4 integrin in conjunctival basement membrane separation: Possible in vitro model for ocular cicatricial pemphigoid. Invest Ophthalmol Vis Sci 1999; 40: 2283-90.
105. Foster CS, Sainz De La Maza M. Ocular cicatricial pemphigoid review. Curr Opin Allergy Clin Immunol 2004; 4: 435-39.
106. Saw VP, Offiah I, Dart RJ, et al. Conjunctival interleukin-13 expression in mucous membrane pemphigoid and functional eff ects of interleukin-13 on conjunctival fi broblasts in vitro. Am J Pathol 2009; 175: 2406-15.
107. Setterfield J, Shirlaw PJ, Kerr-Muir M, et al. Mucous membrane pemphigoid: A dual circulating antibody response with IgG and IgA signifies a more severe and persistent disease. Br J Dermatol 1998; 138: 602-10.
108. Lazarova Z, Sitaru C, Zillikens D, Yancey KB. Comparative analysis of methods for detection of anti-laminin 5 autoantibodies in patients with anti-epiligrin cicatricial pemphigoid. J Am Acad Dermatol 2004; 51: 886-92.
109. Foster CS. Cicatricial pemphigoid. Trans Am Ophthalmol Soc 1986; 84: 527-663.
110. Kirtschig G, Murrell DF, Wojnarowska F, Khumalo NP. Interventions for mucous membrane pemphigoid and epidermolysis bullosa acquisita. Cochrane Database Syst Rev 2009; 4: CD004056.
111. Saw VP, Dart JK, Rauz S, et al. Immunosuppressive therapy for ocular mucous membrane pemphigoid strategies and outcomes. Ophthalmology 2008; 115: 253-61 e1.
112. Letko E, Miserocchi E, Daoud YJ, Christen W, Foster CS, Ahmed AR. A nonrandomized comparison of the clinical outcome of ocular involvement in patients with mucous membrane (cicatricial) pemphigoid between conventional immunosuppressive and intravenous immunoglobulin therapies. Clin Immunol 2004; 111: 303-10.
113. Le Roux-Villet C, Prost-Squarcioni C, Alexandre M, et al. Rituximab for patients with refractory mucous membrane pemphigoid. Arch Dermatol 2011; 147: 843-49.
114. Nanda A, Dvorak R, Al-Saeed K, Al-Sabah H, Alsaleh QA. Spectrum of autoimmune bullous diseases in Kuwait. Int J Dermatol 2004; 43: 876-81.
115. Ambros-Rudolph CM, Mullegger RR, Vaughan-Jones SA, Kerl H, Black MM. The specifi c dermatoses of pregnancy revisited and reclassifi ed: Results of a retrospective two-center study on 505 pregnant patients. J Am Acad Dermatol 2006; 54: 395-404.
116. Jenkins RE, Hern S, Black MM. Clinical features and management of 87 patients with pemphigoid gestationis. Clin Exp Dermatol 1999; 24: 255-59.
117. Intong LR, Murrell DF. Pemphigoid gestationis: Pathogenesis and clinical features. Dermatol Clin 2011; 29: 447-52.
118. 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: 2023-26.
119. Chimanovitch I, Schmidt E, Messer G, et al. IgG1 and IgG3 are the major immunoglobulin subclasses targeting epitopes within the NC16A domain of BP180 in pemphigoid gestationis. J Invest Dermatol 1999; 113: 140-42.
120. Powell AM, Sakuma-Oyama Y, Oyama N, et al. Usefulness of BP180 NC16a enzyme-linked immunosorbent assay in the serodiagnosis of pemphigoid gestationis and in diff erentiating between pemphigoid gestationis and pruritic urticarial papules and plaques of pregnancy. Arch Dermatol 2005; 141: 705-10.
121. Herrero-Gonzalez JE, Brauns O, Egner R, et al. Immunoadsorption against two distinct epitopes on human type XVII collagen abolishes dermal-epidermal separation induced in vitro by autoantibodies from pemphigoid gestationis patients. Eur J Immunol 2006; 36: 1039-48.
122. Chi CC, Wang SH, Charles-Holmes R, et al. Pemphigoid gestationis: Early onset and blister formation are associated with adverse pregnancy outcomes. Br J Dermatol 2009; 160: 1222-28.
123. Wong SN, Chua SH. Spectrum of subepidermal immunobullous disorders seen at the National Skin Centre, Singapore: A 2-year review. Br J Dermatol 2002; 147: 476-80.
124. Wojnarowska F, Marsden RA, Bhogal B, Black MM. Chronic bullous disease of childhood, childhood cicatricial pemphigoid, and linear IgA disease of adults. A comparative study demonstrating clinical and immunopathologic overlap. J Am Acad Dermatol 1988; 19: 792-805.
125. Marinkovich MP, Taylor TB, Keene DR, Burgeson RE, Zone JJ. LAD-1, the linear IgA bullous dermatosis autoantigen, is a novel 120-kDa anchoring fi lament protein synthesized by epidermal cells. J Invest Dermatol 1996; 106: 734-38.
126. Zone JJ, Taylor TB, Kadunce DP, Meyer LJ. Identifi cation of the cutaneous basement membrane zone antigen and isolation of antibody in linear immunoglobulin A bullous dermatosis. J Clin Invest 1990; 85: 812-20.
127. Zillikens D, Herzele K, Georgi M, et al. Autoantibodies in a subgroup of patients with linear IgA disease react with the NC16A domain of BP1801. J Invest Dermatol 1999; 113: 947-53.
128. Zone JJ, Egan CA, Taylor TB, Meyer LJ. IgA autoimmune disorders: Development of a passive transfer mouse model. J Investig Dermatol Symp Proc 2004; 9: 47-51.
129. Ng SY, Venning VV. Management of linear IgA disease. Dermatol Clin 2011; 29: 629-30.
130. Zumelzu C, Le Roux-Villet C, Loiseau P, et al. Black patients of African descent and HLA-DRB1*15:03 frequency overrepresented in epidermolysis bullosa acquisita. J Invest Dermatol 2011; 131: 2386-93.
131. Roenigk HH Jr, Ryan JG, Bergfeld WF. Epidermolysis bullosa acquisita. Report of three cases and review of all published cases. Arch Dermatol 1971; 103: 1-10.
132. Chen M, Kim GH, Prakash L, Woodley DT. Epidermolysis bullosa acquisita: Autoimmunity to anchoring fi bril collagen. Autoimmunity 2012; 45: 91-101.
133. Buijsrogge JJ, Diercks GF, Pas HH, Jonkman MF. The many faces of epidermolysis bullosa acquisita after serration pattern analysis by direct immunofl uorescence microscopy. Br J Dermatol 2011; 165: 92-98.
134. Chen M, O'Toole EA, Sanghavi J, et al. The epidermolysis bullosa acquisita antigen (type VII collagen) is present in human colon and patients with crohn's disease have autoantibodies to type VII collagen. J Invest Dermatol 2002; 118: 1059-64.
135. Woodley DT, Briggaman RA, O'Keefe EJ, Inman AO, Queen LL, Gammon WR. Identifi cation of the skin basement-membrane autoantigen in epidermolysis bullosa acquisita. N Engl J Med 1984; 310: 1007-13.
136. Woodley DT, Burgeson RE, Lunstrum G, Bruckner-Tuderman L, Reese MJ, Briggaman RA. Epidermolysis bullosa acquisita antigen is the globular carboxyl terminus of type VII procollagen. J Clin Invest 1988; 81: 683-87.
137. Chen M, Chan LS, Cai X, O'Toole EA, Sample JC, Woodley DT. Development of an ELISA for rapid detection of anti-type VII collagen autoantibodies in epidermolysis bullosa acquisita. J Invest Dermatol 1997; 108: 68-72.
138. Sitaru C, Mihai S, Otto C, et al. Induction of dermal-epidermal separation in mice by passive transfer of antibodies specifi c to type VII collagen. J Clin Invest 2005; 115: 870-78.
139. Woodley DT, Chang C, Saadat P, Ram R, Liu Z, Chen M. Evidence that anti-type VII collagen antibodies are pathogenic and responsible for the clinical, histological, and immunological features of epidermolysis bullosa acquisita. J Invest Dermatol 2005; 124: 958-64.
140. Sitaru C, Chiriac MT, Mihai S, et al. Induction of complement-fi xing autoantibodies against type VII collagen results in subepidermal blistering in mice. J Immunol 2006; 177: 3461-68.
141. Kasperkiewicz M, Nimmerjahn F, Wende S, et al. Genetic identifi cation and functional validation of FcγRIV as key molecule in autoantibody-induced tissue injury. J Pathol 2012; 228: 8-19.
142. Hirose M, Vafia K, Kalies K, et al. Enzymatic autoantibody glycan hydrolysis alleviates autoimmunity against type VII collagen. J Autoimmun 2012; 39: 304-14.
143. Vodegel RM, Jonkman MF, Pas HH, de Jong MC. U-serrated immunodeposition pattern diff erentiates type VII collagen targeting bullous diseases from other subepidermal bullous autoimmune diseases. Br J Dermatol 2004; 151: 112-18.
144. Saleh MA, Ishii K, Kim YJ, et al. Development of NC1 and NC2 domains of type VII collagen ELISA for the diagnosis and analysis of the time course of epidermolysis bullosa acquisita patients. J Dermatol Sci 2011; 62: 169-75.
145. Komorowski L, Muller R, Vorobyev A, et al. Sensitive and specifi c assays for routine serological diagnosis of epidermolysis bullosa acquisita. J Am Acad Dermatol 2012; published online Feb 15. http://dx.doi.org/10.1016/j.jaad.2011. 12.032.
146. Dainichi T, Koga H, Tsuji T, et al. From anti-p200 pemphigoid to anti-laminin γ1 pemphigoid. J Dermatol 2010; 37: 231-38.
147. Zillikens D, Kawahara Y, Ishiko A, et al. A novel subepidermal blistering disease with autoantibodies to a 200-kDa antigen of the basement membrane zone. J Invest Dermatol 1996; 106: 1333-38.
148. Dainichi T, Kurono S, Ohyama B, et al. Anti-laminin gamma-1 pemphigoid. Proc Natl Acad Sci USA 2009; 106: 2800-05.
149. Groth S, Recke A, Vafia K, et al. Development of a simple ELISA for the detection of autoantibodies in anti-p200 pemphigoid. Br J Dermatol 2011; 164: 76-82.
150. 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 BP180. J Invest Dermatol 1999; 113: 117-21.