Pemphigoid diseases: Pathogenesis, diagnosis, and treatment

References

• Bertram F, Bröcker EB, Zillikens D, Schmidt E. Prospective analysis of the incidence of autoimmune bullous disorders in Lower Franconia, Germany. J Dtsch Dermatol Ges. 2009; 7:434–440.
   PubMed Web of Science ®Google Scholar
• Marazza G, Pham HC, Schärer L, Pedrazzetti PP, Hunziker T, Trüeb RM, Hohl D, Itin P, Lautenschlager S, Naldi L, Borradori L. Autoimmune bullous disease Swiss study group. Incidence of bullous pemphigoid and pemphigus in Switzerland: A 2-year prospective study. Br J Dermatol. 2009; 161:861–868.
   PubMed Web of Science ®Google Scholar
• Cozzani E, Parodi A, Rebora A, Delmonte S, Barile M, Nigro A, Priano L, Troiano G, Patri PL, Gruppo Ligure di Studi in Dermatologia (GLISID). Bullous pemphigoid in Liguria: A 2-year survey. J Eur Acad Dermatol Venereol. 2001; 15:317–319.
   PubMed Web of Science ®Google Scholar
• Gudi VS, White MI, Cruickshank N, Herriot R, Edwards SL, Nimmo F, Ormerod AD. Annual incidence and mortality of bullous pemphigoid in the Grampian Region of North-east Scotland. Br J Dermatol. 2005; 153:424–427.
   PubMed Web of Science ®Google Scholar
• Serwin AB, Bokiniec E, Piascik M, Masny D, Chodynicka B. Epidemiological and clinical analysis of pemphigoid patients in northeastern Poland in 2000–2005. Med Sci Monit. 2007; 13:CR360–CR364.
   PubMed Web of Science ®Google Scholar
• 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::160–163 doi: 10.1136/bmj.a180.
   Google Scholar
• Schmidt E, Zillikens D. Modern diagnosis of autoimmune blistering skin diseases. Autoimmun Rev. 2010; 10:84–89.
   PubMed Web of Science ®Google Scholar
• Stanley JR, Hawley-Nelson P, Yuspa SH, Shevach EM, Katz SI. Characterization of bullous pemphigoid antigen: A unique basement membrane protein of stratified epithelia. Cell. 1981; 24:897–903.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• Labib RS, Anhalt GJ, Patel HP, Mutasim DF, Diaz LA. Molecular heterogeneity of bullous pemphigoid antigens as detected by immunoblotting. J Immunol. 1986; 136:1231–1235.
   PubMed Web of Science ®Google Scholar
• Tanaka M, Hashimoto T, Amagai M, Shimizu N, Ikeguchi N, Tsubata T, Hasegawa A, Miki K, Nishikawa T. Characterization of bullous pemphigoid antibodies by use of recombinant bullous pemphigoid antigen proteins. J Invest Dermatol. 1991; 97:725–728.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• Green KJ, Virata ML, Elgart GW, Stanley JR, Parry DA. Comparative structural analysis of desmoplakin, bullous pemphigoid antigen and plectin: Members of a new gene family involved in organization of intermediate filaments. Int J Biol Macromol. 1992; 14:145–153.
   PubMed Web of Science ®Google Scholar
• Giudice GJ, Emery DJ, Diaz LA. Cloning and primary structural analysis of the bullous pemphigoid autoantigen BP180. J Invest Dermatol. 1992; 99:243–250.
   PubMed Web of Science ®Google Scholar
• Li K, Tamai K, Tan EM, Uitto J, Cloning of type XVII collagen. Complementary and genomic DNA sequences of mouse 180-kilodalton bullous pemphigoid antigen (BPAG2) predict an interrupted collagenous domain, a transmembrane segment, and unusual features in the 5′-end of the gene and the 3′-untranslated region of the mRNA. J Biol Chem. 1993; 268:8825–8834.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• 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. 1997a; 109:573–579.
   PubMed Web of Science ®Google Scholar
• Zillikens D, Mascaro JM, Rose PA, Liu Z, Ewing SM, Caux F, Hoffmann RG, Diaz LA, Giudice GJ. A highly sensitive enzyme-linked immunosorbent assay for the detection of circulating anti-BP180 autoantibodies in patients with bullous pemphigoid. J Invest Dermatol. 1997b; 109:679–683.
   PubMed Web of Science ®Google Scholar
• Sitaru C, Dähnrich C, Probst C, Komorowski L, Blöcker I, Schmidt E, Schlumberger W, Rose C, Stöcker W, Zillikens D. Enzyme-linked immunosorbent assay using multimers of the 16th non-collagenous domain of the BP180 antigen for sensitive and specific detection of pemphigoid autoantibodies. Exp Dermatol. 2007; 16:770–777.
   PubMed Web of Science ®Google Scholar
• Schmidt E, Obe K, Bröcker EB, Zillikens D. Serum levels of autoantibodies to BP180 correlate with disease activity in patients with bullous pemphigoid. Arch Dermatol. 2000; 136:174–178.
   PubMed Web of Science ®Google Scholar
• Zimina EP, Fritsch A, Schermer B, Bakulina AY, Bashkurov M, Benzing T, Bruckner-Tuderman L. Extracellular phosphorylation of collagen XVII by ecto-casein kinase 2 inhibits ectodomain shedding. J Biol Chem. 2007; 282:22737–22746.
   PubMed Web of Science ®Google Scholar
• Zimina EP, Hofmann SC, Fritsch A, Kern JS, Sitaru C, Bruckner-Tuderman L. Bullous pemphigoid autoantibodies preferentially recognize phosphoepitopes in collagen XVII. J Invest Dermatol. 2008; 128:2736–2739.
   PubMed Web of Science ®Google Scholar
• Leyendeckers H, Tasanen K, Bruckner-Tuderman L, Zillikens D, Sitaru C, Schmitz J, Hunzelmann N. Memory B cells specific for the NC16A domain of the 180 kDa bullous pemphigoid autoantigen can be detected in peripheral blood of bullous pemphigoid patients and induced in vitro to synthesize autoantibodies. J Invest Dermatol. 2003; 120:372–378.
   PubMed Web of Science ®Google Scholar
• László S, Neumann S, Hertl M, Hunzelmann N. Generation of increased numbers of HLA-DR(high) IgG+ plasma cells in the peripheral blood of patients with bullous pemphigoid: NC16a-specific cells belong to the short-lived plasma blast population. J Invest Dermatol. 2010; 130:2838–2841.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMedGoogle Scholar
• Döpp R, Schmidt E, Chimanovitch I, Leverkus M, Bröcker 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.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• Dimson OG, Giudice GJ, Fu CL, Van den Bergh F, Warren SJ, Janson MM, Fairley JA. Identification of a potential effector function for IgE autoantibodies in the organ-specific autoimmune disease bullous pemphigoid. J Invest Dermatol. 2003; 120:784–788.
   PubMed Web of Science ®Google Scholar
• Iwata Y, Komura K, Kodera M, Usuda T, Yokoyama Y, Hara T, Muroi E, Ogawa F, Takenaka M, Sato S. Correlation of IgE autoantibody to BP180 with a severe form of bullous pemphigoid. Arch Dermatol. 2008; 144:41–48.
   PubMedGoogle Scholar
• Fairley JA, Fu CL, Giudice GJ. Mapping the binding sites of anti-BP180 immunoglobulin E autoantibodies in bullous pemphigoid. J Invest Dermatol. 2005; 125:467–472.
   PubMed Web of Science ®Google Scholar
• Giudice GJ, Wilske KC, Anhalt GJ, Fairley JA, Taylor AF, Emery DJ, Hoffman RG, Diaz LA. Development of an ELISA to detect anti-BP180 autoantibodies in bullous pemphigoid and herpes gestationis. J Invest Dermatol. 1994; 102:878–881.
   PubMed Web of Science ®Google Scholar
• Perriard J, Jaunin F, Favre B, Büdinger L, Hertl M, Saurat JH, Borradori L. 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; 112:141–147.
   PubMed Web of Science ®Google Scholar
• Di Zenzo G, Grosso F, Terracina M, Mariotti F, De Pità O, Owaribe K, Mastrogiacomo A, Sera F, Borradori L, Zambruno G. Characterization of the anti-BP180 autoantibody reactivity profile and epitope mapping in bullous pemphigoid patients. J Invest Dermatol. 2004; 122:103–110.
   PubMed Web of Science ®Google Scholar
• Dresow SK, Sitaru C, Recke A, Oostingh GJ, Zillikens D, Gibbs BF. IgE autoantibodies against the intracellular domain of BP180. Br J Dermatol. 2009; 160:429–432.
   PubMed Web of Science ®Google Scholar
• Skaria M, Jaunin F, Hunziker T, Riou S, Schumann H, Bruckner-Tuderman L, Hertl M, Bernard P, Saurat JH, Favre B, Borradori L. 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.
   PubMed Web of Science ®Google Scholar
• Kromminga A, Sitaru C, Hagel C, Herzog S, Zillikens D. Development of an ELISA for the detection of autoantibodies to BP230. Clin Immunol. 2004; 111:146–152.
   PubMed Web of Science ®Google Scholar
• Thoma-Uszynski S, Uter W, Schwietzke S, Hofmann SC, Hunziker T, Bernard P, Treudler R, Zouboulis CC, Schuler G, Borradori L, Hertl M. BP230- and BP180-specific auto-antibodies in bullous pemphigoid. J Invest Dermatol. 2004; 122:1413–1422.
   PubMed Web of Science ®Google Scholar
• Di Zenzo G, Thoma-Uszynski S, Fontao L, Calabresi V, Hofmann SC, Hellmark T, Sebbag N, Pedicelli C, Sera F, Lacour JP, Wieslander J, Bruckner-Tuderman L, Borradori L, Zambruno G, Hertl M. Multicenter prospective study of the humoral autoimmune response in bullous pemphigoid. Clin Immunol. 2008; 128:415–426.
   PubMed Web of Science ®Google Scholar
• Charneux J, Lorin J, Vitry F, Antonicelli F, Reguiai Z, Barbe C, Tabary T, Grange F, Bernard P. 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; 147:286–291.
   PubMedGoogle Scholar
• Roussel A, Benichou J, Randriamanantany ZA, Gilbert D, Drenovska K, Houivet E, Tron F, Joly P. Enzyme-linked immunosorbent assay for the combination of bullous pemphigoid antigens 1 and 2 in the diagnosis of bullous pemphigoid. Arch Dermatol. 2011; 147:293–298.
   PubMedGoogle Scholar
• 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. 2015; 176:2015–2023.
   Google Scholar
• Büdinger 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.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• Schmidt E, Ambach A, Bastian B, Bröcker EB, Zillikens D. Elevated levels of interleukin-8 in blister fluid of bullous pemphigoid compared with suction blisters of healthy control subjects. J Am Acad Dermatol. 1996; 34:310–312.
   PubMed Web of Science ®Google Scholar
• Schmidt E, Bastian B, Dummer R, Tony HP, Bröcker EB, Zillikens D. Detection of elevated levels of IL-4, IL-6, and IL-10 in blister fluid of bullous pemphigoid. Arch Dermatol Res. 1996; 288:353–357.
   PubMed Web of Science ®Google Scholar
• Schmidt E, Mittnacht A, Schomig H, Dummer R, Bröcker EB, Zillikens D. Detection of IL-1 alpha, IL-1 beta and IL-1 receptor antagonist in blister fluid of bullous pemphigoid. J Dermatol Sci. 1996; 11:142–147.
   PubMed Web of Science ®Google Scholar
• Ameglio F, D'Auria L, Bonifati C, Ferraro C, Mastroianni A, Giacalone B. Cytokine pattern in blister fluid and serum of patients with bullous pemphigoid: Relationships with disease intensity. Br J Dermatol. 1998; 138:611–614.
   PubMed Web of Science ®Google Scholar
• Inaoki M, Takehara K. Increased serum levels of interleukin (IL)-5, IL-6 and IL-8 in bullous pemphigoid. J Dermatol Sci. 1998; 16:152–157.
   PubMed Web of Science ®Google Scholar
• D'Auria L, Bonifati C, Cordiali-Fei P, Leone G, Picardo M, Pietravalle M, Giacalone B, Ameglio F. Increased serum interleukin-15 levels in bullous skin diseases: Correlation with disease intensity. Arch Dermatol Res. 1999; 291:354–356.
   PubMed Web of Science ®Google Scholar
• Wakugawa W, 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.
   PubMed Web of Science ®Google Scholar
• Frezzolini A, Teofoli P, Cianchini G, Barduagni S, Ruffelli M, Ferranti G, Puddu P, Pita OD. Increased expression of eotaxin and its specific receptor CCR3 in bullous pemphigoid. Eur J Dermatol. 2002; 12:27–31.
   PubMed Web of Science ®Google Scholar
• Frezzolini A, Cianchini G, Ruffelli M, Cadoni S, Puddu P, De Pita O. Interleukin-16 expression and release in bullous pemphigoid. Clin Exp Immunol. 2004; 137:595–600.
   PubMed Web of Science ®Google Scholar
• Gounni Abdelilah S, Wellemans V, Agouli M, Guenounou M, Hamid Q, Beck LA, Lamkhioued B. Increased expression of Th2-associated chemokines in bullous pemphigoid disease: Role of eosinophils in the production and release of these chemokines. Clin Immunol. 2006; 120:220–231.
   PubMed Web of Science ®Google Scholar
• Günther C, Carballido-Perrig N, Kopp T, Carballido JM, Pfeiffer C. CCL18 is expressed in patients with bullous pemphigoid and parallels disease course. Br J Dermatol. 2009; 160:747–755.
   PubMed Web of Science ®Google Scholar
• Ludwig RJ, Schmidt E. Cytokines in autoimmune bullous skin diseases: Epiphenomena or contribution to pathogenesis?. G Ital Dermatol Venereol. 2009; 144:339–349.
   PubMed Web of Science ®Google Scholar
• Teraki Y, Hotta T, Shiohara T. Skin-homing interleukin-4 and -13-producing cells contribute to bullous pemphigoid: Remission of disease is associated with increased frequency of interleukin-10-producing cells. J Invest Dermatol. 2001; 117:1097–1102.
   PubMed Web of Science ®Google Scholar
• Rensing-Ehl A, Gaus B, Bruckner-Tuderman L, Martin SF. Frequency, function and CLA expression of CD4+CD25+FOXP3+ regulatory T cells in bullous pemphigoid. Exp Dermatol. 2007; 16:13–21.
   PubMed Web of Science ®Google Scholar
• Oswald E, Fisch P, Jakob T, Bruckner-Tuderman L, Martin SF, Rensing-Ehl A. Reduced numbers of circulating gammadelta T cells in patients with bullous pemphigoid. Exp Dermatol. 2009; 18:991–993.
   PubMed Web of Science ®Google Scholar
• Kasperkiewicz M, Zillikens D. The pathophysiology of bullous pemphigoid. Clin Rev Allergy Immunol. 2007; 33:67–77.
   PubMed Web of Science ®Google Scholar
• Bieber K, Sun S, Ishii N, Kasperkiewicz M, Schmidt E, Hirose M, Westermann J, Yu X, Zillikens D, Ludwig RJ. Animal models for autoimmune bullous dermatoses. Exp Dermatol. 2010; 19:2–11.
   PubMed Web of Science ®Google Scholar
• Schmidt E, Reimer S, Kruse N, Jainta S, Bröcker EB, Marinkovich MP, Giudice GJ, Zillikens D. Autoantibodies to BP180 associated with bullous pemphigoid release interleukin-6 and interleukin-8 from cultured human keratinocytes. J Invest Dermatol. 2000; 115:842–848.
   PubMed Web of Science ®Google Scholar
• Iwata H, Kamio N, Aoyama Y, Yamamoto Y, Hirako Y, Owaribe K, Kitajima Y. 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; 129:919–926.
   PubMed Web of Science ®Google Scholar
• Gammon WR, Merritt CC, Lewis DM, Sams WMJr, Carlo JR, Wheeler CEJr. An in vitro model of immune complex-mediated basement membrane zone separation caused by pemphigoid antibodies, leukocytes, and complement. J Invest Dermatol. 1982; 78:285–290.
   PubMed Web of Science ®Google Scholar
• Sitaru C, Schmidt E, Petermann S, Munteanu LS, Bröcker 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.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• Welgus HG, Bauer EA, Stricklin GP. Elevated levels of human collagenase inhibitor in blister fluids of diverse etiology. J Invest Dermatol. 1986; 87:592–596.
   PubMed Web of Science ®Google Scholar
• Jensen PJ, Baird J, Morioka S, Lessin S, Lazarus GS. Epidermal plasminogen activator is abnormal in cutaneous lesions. J Invest Dermatol. 1988; 90:777–782.
   PubMed Web of Science ®Google Scholar
• Grando SA, Glukhenky BT, Drannik GN, Kostromin AP, Chernyavsky AI. Cytotoxic proteases in blister fluid of pemphigus and pemphigoid patients. Int J Tissue React. 1989; 11:195–201.
   PubMedGoogle Scholar
• Grando SA, Glukhenky BT, Drannik GN, Epshtein EV, Kostromin AP, Korostash TA. Mediators of inflammation in blister fluids from patients with pemphigus vulgaris and bullous pemphigoid. Arch Dermatol. 1989; 125:925–930.
   PubMed Web of Science ®Google Scholar
• Gissler HM, Simon MM, Kramer MD. Enhanced association of plasminogen/plasmin with lesional epidermis of bullous pemphigoid. Br J Dermatol. 1992; 127:272–277.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• Niimi Y, Pawankar R, Kawana S. Increased expression of matrix metalloproteinase-2, matrix metalloproteinase-9 and matrix metalloproteinase-13 in lesional skin of bullous pemphigoid. Int Arch Allergy Immunol. 2006; 139:104–113.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• Shimanovich I, Mihai S, Oostingh GJ, Ilenchuk TT, Bröcker EB, Opdenakker G, Zillikens D, Sitaru C. Granulocyte-derived elastase and gelatinase B are required for dermal-epidermal separation induced by autoantibodies from patients with epidermolysis bullosa acquisita and bullous pemphigoid. J Pathol. 2004; 204:519–527.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• Liu Z, Li N, Diaz LA, Shipley M, Senior RM, Werb Z. Synergy between a plasminogen cascade and MMP-9 in autoimmune disease. J Clin Invest. 2005; 115:879–887.
   PubMed Web of Science ®Google Scholar
• Schmidt E, Wehr B, Tabengwa EM, Reimer S, Bröcker EB, Zillikens D. Elevated expression and release of tissue-type, but not urokinase-type, plasminogen activator after binding of autoantibodies to bullous pemphigoid antigen 180 in cultured human keratinocytes. Clin Exp Immunol. 2004; 135:497–504.
   PubMed Web of Science ®Google Scholar
• Coussens LM, Raymond WW, Bergers G, Laig-Webster M, Behrendtsen O, Werb Z, Caughey GH, Hanahan D. Inflammatory mast cells up-regulate angiogenesis during squamous epithelial carcinogenesis. Genes Dev. 1999; 13:1382–1397.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• Zillikens D, Schmidt E, Reimer S, Chimanovitch I, Hardt-Weinelt K, Rose C, Bröcker EB, Kock M, Boehncke WH. Antibodies to desmogleins 1 and 3, but not to BP180, induce blisters in human skin grafted onto SCID mice. J Pathol. 2001; 193:117–124.
   PubMed Web of Science ®Google Scholar
• Olasz EB, Roh J, Yee CL, Arita K, Akiyama M, Shimizu H, Vogel JC, Yancey KB. Human bullous pemphigoid antigen 2 transgenic skin elicits specific IgG in wild-type mice. J Invest Dermatol. 2007; 127:2807–2817.
   PubMed Web of Science ®Google Scholar
• Nishie W, Sawamura D, Goto M, Ito K, Shibaki A, McMillan JR, Sakai K, Nakamura H, Olasz E, Yancey KB, Akiyama M, Shimizu H. Humanization of autoantigen. Nat Med. 2007; 13:378–383.
   PubMed Web of Science ®Google Scholar
• Li Q, Ujiie H, Shibaki A, Wang G, Moriuchi R, Qiao HJ, Morioka H, Shinkuma S, Natsuga K, Long HA, Nishie W, Shimizu H. Human IgG1 monoclonal antibody against human collagen 17 NC16A induces blisters via complement activation in experimental bullous pemphigoid model. J Immunol. 2010; 185:7746–7755.
   PubMed Web of Science ®Google Scholar
• Liu Z, Sui W, Zhao M, Li Z, Li N, Thresher R, Giudice GJ, Fairley JA, Sitaru C, Zillikens D, Ning G, Marinkovich MP, Diaz LA. Subepidermal blistering induced by human autoantibodies to BP180 requires innate immune players in a humanized bullous pemphigoid mouse model. J Autoimmun. 2008; 31:331–338.
   PubMed Web of Science ®Google Scholar
• Ujiie H, Shibaki A, Nishie W, Sawamura D, Wang G, Tateishi Y, Li Q, Moriuchi R, Qiao H, Nakamura H, Akiyama M, Shimizu H. A novel active mouse model for bullous pemphigoid targeting humanized pathogenic antigen. J Immunol. 2010; 184:2166–2174.
   PubMed Web of Science ®Google Scholar
• Zone JJ, Taylor T, Hull C, Schmidt L, Meyer L. IgE basement membrane zone antibodies induce eosinophil infiltration and histological blisters in engrafted human skin on SCID mice. J Invest Dermatol. 2007; 127:1167–1174.
   PubMed Web of Science ®Google Scholar
• 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; 127:2605–2611.
   PubMed Web of Science ®Google Scholar
• Joly P, Benichou J, Lok C, Hellot MF, Saiag P, Tancrede-Bohin E, Sassolas B, Labeille B, Doutre MS, Gorin I, Pauwels C, Chosidow O, Caux F, Estève E, Dutronc Y, Sigal M, Prost C, Maillard H, Guillaume JC, Roujeau JC. Prediction of survival for patients with bullous pemphigoid: A prospective study. Arch Dermatol. 2005; 141:691–698.
   PubMedGoogle Scholar
• Parker SR, Dyson S, Brisman S, Pennie M, Swerlick RA, Khan R, Manos S, Korman BD, Xia Z, Korman NJ. Mortality of bullous pemphigoid: An evaluation of 223 patients and comparison with the mortality in the general population in the United States. J Am Acad Dermatol. 2008; 59:582–588.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• Kobayashi M, Amagai M, Kuroda-Kinoshita K, Hashimoto T, Shirakata Y, Hashimoto K, Nishikawa T. BP180 ELISA using bacterial recombinant NC16a protein as a diagnostic and monitoring tool for bullous pemphigoid. J Dermatol Sci. 2002; 30:224–232.
   PubMed Web of Science ®Google Scholar
• Hofmann S, Thoma-Uszynski S, Hunziker T, Bernard P, Koebnick C, Stauber A, Schuler G, Borradori L, Hertl M. 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; 119:1065–1073.
   PubMed Web of Science ®Google Scholar
• Yoshida M, Hamada T, Amagai M, Hashimoto K, Uehara R, Yamaguchi K, Imamura K, Okamoto E, Yasumoto S, Hashimoto T. 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.
   PubMed Web of Science ®Google Scholar
• Vodegel RM, Jonkman MF, Pas HH, de Jong MC. U-serrated immunodeposition pattern differentiates type VII collagen targeting bullous diseases from other subepidermal bullous autoimmune diseases. Br J Dermatol. 2004; 151:112–118.
   PubMed Web of Science ®Google Scholar
• Roujeau JC, Lok C, Bastuji-Garin S, Mhalla S, Enginger V, Bernard P. High risk of death in elderly patients with extensive bullous pemphigoid. Arch Dermatol. 1998; 134:465–469.
   PubMedGoogle Scholar
• Rzany B, Partscht K, Jung M, Kippes W, Mecking D, Baima B, Prudlo C, Pawelczyk B, Messmer EM, Schuhmann M, Sinkgraven R, Büchner L, Büdinger L, Pfeiffer C, Sticherling M, Hertl M, Kaiser HW, Meurer M, Zillikens D, Messer G. Risk factors for lethal outcome in patients with bullous pemphigoid: Low serum albumin level, high dosage of glucocorticosteroids, and old age. Arch Dermatol. 2002; 138:903–908.
   PubMedGoogle Scholar
• Colbert RL, Allen DM, Eastwood D, Fairley JA. Mortality rate of bullous pemphigoid in a US medical center. J Invest Dermatol. 2004; 122:1091–1095.
   PubMed Web of Science ®Google Scholar
• Kirtschig G, Middleton P, Bennett C, Murrell DF, Wojnarowska F, Khumalo NP. Interventions for bullous pemphigoid. Cochrane Database Syst Rev. 2010 CD002292.
   PubMed Web of Science ®Google Scholar
• 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; 111:925–928.
   PubMed Web of Science ®Google Scholar
• Joly P, Roujeau JC, Benichou J, Picard C, Dreno B, Delaporte E, Vaillant L, D'Incan M, Plantin P, Bedane C, Young P, Bernard P, Bullous Diseases French Study Group. A comparison of oral and topical corticosteroids in patients with bullous pemphigoid. N Engl J Med. 2002; 346:321–327.
   PubMed Web of Science ®Google Scholar
• Joly P, Roujeau JC, Benichou J, Delaporte E, D'Incan M, Dreno B, Bedane C, Sparsa A, Gorin I, Picard C, Tancrede-Bohin E, Sassolas B, Lok C, Guillaume JC, Doutre MS, Richard MA, Caux F, Prost C, Plantin P, Chosidow O, Pauwels C, Maillard H, Saiag P, Descamps V, Chevrant-Breton J, Dereure O, Hellot MF, Esteve E, Bernard P. A comparison of two regimens of topical corticosteroids in the treatment of patients with bullous pemphigoid: A multicenter randomized study. J Invest Dermatol. 2009 July; 129 7: 1681–1687.
   PubMed Web of Science ®Google Scholar
• Guillaume JC, Vaillant L, Bernard P, Picard C, Prost C, Labeille B, Guillot B, Foldès-Pauwels C, Prigent F, Joly P, Cordoliani F, Salagnac V, Machet L, Aldigier JC, Crickx B, Roujeau JC. Controlled trial of azathioprine and plasma exchange in addition to prednisolone in the treatment of bullous pemphigoid. Arch Dermatol. 1993; 129:49–53.
   PubMedGoogle Scholar
• Beissert S, Werfel T, Frieling U, Böhm M, Sticherling M, Stadler R, Zillikens D, Rzany B, Hunzelmann N, Meurer M, Gollnick H, Ruzicka T, Pillekamp H, Junghans V, Bonsmann G, Luger TA. A comparison of oral methylprednisolone plus azathioprine or mycophenolate mofetil for the treatment of bullous pemphigoid. Arch Dermatol. 2007; 143:1536–1542.
   PubMed Web of Science ®Google Scholar
• Fivenson DP, Breneman DL, Rosen GB, Hersh CS, Cardone S, Mutasim D. Nicotinamide and tetracycline therapy of bullous pemphigoid. Arch Dermatol. 1994; 130:753–758.
   PubMedGoogle Scholar
• Schmidt E, Kraensel R, Goebeler M, Sinkgraven R, Bröcker EB, Rzany B, Zillikens D. Treatment of bullous pemphigoid with dapsone, methylprednisolone, and topical clobetasol propionate: A retrospective study of 62 cases. Cutis. 2005; 76:205–209.
   PubMed Web of Science ®Google Scholar
• Gürcan HM, Ahmed AR. Efficacy of dapsone in the treatment of pemphigus and pemphigoid: Analysis of current data. Am J Clin Dermatol. 2009; 10:383–396.
   PubMed Web of Science ®Google Scholar
• Gürcan HM, Ahmed AR. Analysis of current data on the use of methotrexate in the treatment of pemphigus and pemphigoid. Br J Dermatol. 2009; 161:723–731.
   PubMed Web of Science ®Google Scholar
• Ishii N, Hashimoto T, Zillikens D, Ludwig RJ. High-dose intravenous immunoglobulin (IVIG) therapy in autoimmune skin blistering diseases. Clin Rev Allergy Immunol. 2010; 38:186–195.
   PubMed Web of Science ®Google Scholar
• Schmidt E, Bröcker EB, Goebeler M. Rituximab in treatment-resistant autoimmune blistering skin disorders. Clin Rev Allergy Immunol. 2008; 34:56–64.
   PubMed Web of Science ®Google Scholar
• Hertl M, Zillikens D, Borradori L, Bruckner-Tuderman L, Burckhard H, Eming R, Engert A, Goebeler M, Hofmann S, Hunzelmann N, Karlhofer F, Kautz O, Lippert U, Niedermeier A, Nitschke M, Pfütze M, Reiser M, Rose C, Schmidt E, Shimanovich I, Sticherling M, Wolff-Franke S. Recommendations for the use of rituximab (anti-CD20 antibody) in the treatment of autoimmune bullous skin diseases. J Dtsch Dermatol Ges. 2008; 6:366–373.
   PubMed Web of Science ®Google Scholar
• Kasperkiewicz M, Shimanovich I, Ludwig RJ, Rose C, Zillikens D, Schmidt E. Rituximab for treatment-refractory pemphigus and pemphigoid: A case series of 17 patients. J Am Acad Dermatol. 2011; 65:552–558.
   PubMed Web of Science ®Google Scholar
• 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; 123:704–705.
   PubMed Web of Science ®Google Scholar
• Schmidt E, Zillikens D. Immunoadsorption in dermatology. Arch Dermatol Res. 2010; 302:241–253.
   PubMed Web of Science ®Google Scholar
• Schmidt E, Skrobek C, Kromminga A, Hashimoto T, Messer G, Bröcker EB, Yancey KB, Zillikens D. Cicatricial pemphigoid: IgA and IgG autoantibodies target epitopes on both intra- and extracellular domains of bullous pemphigoid antigen 180. Br J Dermatol. 2001; 145:778–783.
   PubMed Web of Science ®Google Scholar
• Bédane C, McMillan JR, Balding SD, Bernard P, Prost C, Bonnetblanc JM, Diaz LA, Eady RA, Giudice GJ. Bullous pemphigoid and cicatricial pemphigoid autoantibodies react with ultrastructurally separable epitopes on the BP180 ectodomain: Evidence that BP180 spans the lamina lucida. J Invest Dermatol. 1997; 108:901–907.
   PubMed Web of Science ®Google Scholar
• Oyama N, Setterfield JF, Powell AM, Sakuma-Oyama Y, Albert S, Bhogal BS, Vaughan RW, Kaneko F, Challacombe SJ, Black MM. 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.
   PubMed Web of Science ®Google Scholar
• Bernard P, Prost C, Lecerf V, Intrator L, Combemale P, Bedane C, Roujeau JC, Revuz J, Bonnetblanc JM, Dubertret L. Studies of cicatricial pemphigoid autoantibodies using direct immunoelectron microscopy and immunoblot analysis. J Invest Dermatol. 1990; 94:630–635.
   PubMed Web of Science ®Google Scholar
• Calabresi V, Carrozzo M, Cozzani E, Arduino P, Bertolusso G, Tirone F, Parodi A, Zambruno G, Di Zenzo G. Oral pemphigoid autoantibodies preferentially target BP180 ectodomain. Clin Immunol. 2007; 122:207–213.
   PubMed Web of Science ®Google Scholar
• 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; 90:1628–1633.
   PubMed Web of Science ®Google Scholar
• Chan LS, Majmudar AA, Tran HH, Meier F, Schaumburg-Lever G, Chen M, Anhalt G, Woodley DT, Marinkovich MP. Laminin-6 and laminin-5 are recognized by autoantibodies in a subset of cicatricial pemphigoid. J Invest Dermatol. 1997; 108:848–853.
   PubMed Web of Science ®Google Scholar
• Luke MC, Darling TN, Hsu R, Summers RM, Smith JA, Solomon BI, Thomas GR, Yancey KB. Mucosal morbidity in patients with epidermolysis bullosa acquisita. Arch Dermatol. 1999; 135:954–959.
   PubMedGoogle Scholar
• Tyagi S, Bhol K, Natarajan K, Livir-Rallatos C, Foster CS, Ahmed AR. Ocular cicatricial pemphigoid antigen: Partial sequence and biochemical characterization. Proc Natl Acad Sci USA. 1996; 93:14714–14719.
   PubMed Web of Science ®Google Scholar
• Rashid KA, Gürcan HM, Ahmed AR. Antigen specificity in subsets of mucous membrane pemphigoid. J Invest Dermatol. 2006; 126:2631–2636.
   PubMed Web of Science ®Google Scholar
• 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; 98:1509–1518.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• 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; 114:178–184.
   PubMed Web of Science ®Google Scholar
• Razzaque MS, Foster CS, Ahmed AR. Tissue and molecular events in human conjunctival scarring in ocular cicatricial pemphigoid. Histol Histopathol. 2001; 16:1203–1212.
   PubMed Web of Science ®Google Scholar
• Saw VP, Schmidt E, Offiah I, Galatowicz G, Zillikens D, Dart JK, Calder VL, Daniels JT. Profibrotic phenotype of conjunctival fibroblasts from mucous membrane pemphigoid. Am J Pathol. 2011; 178:187–197.
   PubMed Web of Science ®Google Scholar
• Chan LS, Ahmed AR, Anhalt GJ, Bernauer W, Cooper KD, Elder MJ, Fine JD, Foster CS, Ghohestani R, Hashimoto T, Hoang-Xuan T, Kirtschig G, Korman NJ, Lightman S, Lozada-Nur F, Marinkovich MP, Mondino BJ, Prost-Squarcioni C, Rogers RS3rd, Setterfield JF, West DP, Wojnarowska F, Woodley DT, Yancey KB, Zillikens D, Zone JJ. The first international consensus on mucous membrane pemphigoid: Definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol. 2002; 138:370–379.
   PubMed Web of Science ®Google Scholar
• Rose C, Schmidt E, Kerstan A, Thoma-Uszynski S, Wesselmann U, Käsbohrer U, Zillikens D, Shimanovich I. Histopathology of anti-laminin 5 mucous membrane pemphigoid. J Am Acad Dermatol. 2009; 61:433–440.
   PubMed Web of Science ®Google Scholar
• Horváth B, Niedermeier A, Podstawa E, Müller R, Hunzelmann N, Kárpáti S, Hertl M. IgA autoantibodies in the pemphigoids and linear IgA bullous dermatosis. Exp Dermatol. 2010; 19:648–653.
   PubMed Web of Science ®Google Scholar
• Leverkus M, Schmidt E, Lazarova Z, Bröcker EB, Yancey KB, Zillikens D. Antiepiligrin cicatricial pemphigoid: An underdiagnosed entity within the spectrum of scarring autoimmune subepidermal bullous diseases?. Arch Dermatol. 1999; 135:1091–1098.
   PubMedGoogle Scholar
• Egan CA, Lazarova Z, Darling TN, Yee C, Coté T, Yancey KB. Anti-epiligrin cicatricial pemphigoid and relative risk for cancer. Lancet. 2001; 357:1850–1851.
   PubMed Web of Science ®Google Scholar
• 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; 51:886–892.
   PubMed Web of Science ®Google Scholar
• Brunsting LA, Perry HO. Benign pemphigoid? A report of seven cases with chronic, scarring, herpetiform plaques about the head and neck. Arch Dermatol. 1957; 75:489–501.
   Google Scholar
• Kurzhals G, Stolz W, Meurer M, Kunze J, Braun-Falco O, Krieg T. Acquired epidermolysis bullosa with the clinical feature of Brunsting–Perry cicatricial bullous pemphigoid. Arch Dermatol. 1991; 127:391–395.
   PubMedGoogle Scholar
• Daito J, Katoh N, Asai J, Ueda E, Takenaka H, Ishii N, Hashimoto T, Kishimoto S. Brunsting–Perry cicatricial pemphigoid associated with autoantibodies to the C-terminal domain of BP180. Br J Dermatol. 2008; 159:984–986.
   PubMed Web of Science ®Google Scholar
• Jedlickova H, Niedermeier A, Zgažarová S, Hertl M. Brunsting–Perry pemphigoid of the scalp with antibodies against laminin 332. Dermatology. 2011; 222:193–195.
   PubMed Web of Science ®Google Scholar
• Meyer-ter-Vehn T, Schmidt E, Zillikens D, Geerling G. Mucous membrane pemphigoid with ocular involvement. Part II: Therapy. Ophthalmologe. 2008; 105:405–419.
   PubMed Web of Science ®Google Scholar
• Knudson RM, Kalaaji AN, Bruce AJ. The management of mucous membrane pemphigoid and pemphigus. Dermatol Ther. 2010; 23:268–280.
   PubMed Web of Science ®Google Scholar
• Sami N, Letko E, Androudi S, Daoud Y, Foster CS, Ahmed AR. Intravenous immunoglobulin therapy in patients with ocular-cicatricial pemphigoid: A long-term follow-up. Ophthalmology. 2004; 111:1380–1382.
   PubMed Web of Science ®Google Scholar
• O'Neill ID. Off-label use of biologicals in the management of inflammatory oral mucosal disease. J Oral Pathol Med. 2008; 37:575–581.
   PubMed Web of Science ®Google Scholar
• Shimanovich I, Bröcker EB, Zillikens D. Pemphigoid gestationis: New insights into the pathogenesis lead to novel diagnostic tools. BJOG. 2002; 109:970–976.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• Murakami H, Amagai M, Higashiyama M, Hashimoto K, Chorzelski TP, Bhogal BS, Jenkins RE, Black MM, Zillikens D, Nishikawa T, Hashimoto T. Analysis of antigens recognized by autoantibodies in herpes gestationis: Usefulness of immunoblotting using a fusion protein representing an extracellular domain of the 180 kD bullous pemphigoid antigen. J Dermatol Sci. 1996; 13:112–117.
   PubMed Web of Science ®Google Scholar
• Chimanovitch I, Schmidt E, Messer G, Döpp R, Partscht K, Bröcker EB, Giudice GJ, Zillikens D. IgG1 and IgG3 are the major immunoglobulin subclasses targeting epitopes within the NC16A domain of BP180 in pemphigoid gestationis. J Invest Dermatol. 1999; 113:140–142.
   PubMed Web of Science ®Google Scholar
• Di Zenzo G, Calabresi V, Grosso F, Caproni M, Ruffelli M, Zambruno G. The intracellular and extracellular domains of BP180 antigen comprise novel epitopes targeted by pemphigoid gestationis autoantibodies. J Invest Dermatol. 2007; 127:864–873.
   PubMed Web of Science ®Google Scholar
• Herrero-González JE, Brauns O, Egner R, Rönspeck W, Mascaró JMJr, Jonkman MF, Zillikens D, Sitaru C. 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; 36:1039–1048.
   PubMed Web of Science ®Google Scholar
• Döpp R, Schmidt E, Chimanovitch I, Leverkus M, Bröcker 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.
   PubMed Web of Science ®Google Scholar
• Lin MS, Gharia MA, Swartz SJ, Diaz LA, Giudice GJ. Identification and characterization of epitopes recognized by T lymphocytes and autoantibodies from patients with herpes gestationis. J Immunol. 1999; 162:4991–4997.
   PubMed Web of Science ®Google Scholar
• Sitaru C, Powell J, Messer G, Bröcker EB, Wojnarowska F, Zillikens D. Immunoblotting and enzyme-linked immunosorbent assay for the diagnosis of pemphigoid gestationis. Obstet Gynecol. 2004; 103:757–763.
   PubMed Web of Science ®Google Scholar
• Wojnarowska F, Whitehead P, Leigh IM, Bhogal BS, Black MM. Identification of the target antigen in chronic bullous disease of childhood and linear IgA disease of adults. Br J Dermatol. 1991; 124:157–162.
   PubMed Web of Science ®Google Scholar
• Marinkovich MP, Taylor TB, Keene DR, Burgeson RE, Zone JJ. LAD-1, the linear IgA bullous dermatosis autoantigen, is a novel 120-kDa anchoring filament protein synthesized by epidermal cells. J Invest Dermatol. 1996; 106:734–738.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMed Web of Science ®Google Scholar
• Zone JJ, Taylor TB, Kadunce DP, Meyer LJ. Identification of the cutaneous basement membrane zone antigen and isolation of antibody in linear immunoglobulin A bullous dermatosis. J Clin Invest. 1990; 85:812–820.
   PubMed Web of Science ®Google Scholar
• Pas HH, Kloosterhuis GJ, Heeres K, van der Meer JB, Jonkman MF. Bullous pemphigoid and linear IgA dermatosis sera recognize a similar 120-kDa keratinocyte collagenous glycoprotein with antigenic cross-reactivity to BP180. J Invest Dermatol. 1997; 108:423–429.
   PubMed Web of Science ®Google Scholar
• Hirako Y, Usukura J, Uematsu J, Hashimoto T, Kitajima Y, Owaribe K. Cleavage of BP180, a 180-kDa bullous pemphigoid antigen, yields a 120-kDa collagenous extracellular polypeptide. J Biol Chem. 1998; 273:9711–9717.
   PubMed Web of Science ®Google Scholar
• Schäcke H, Schumann H, Hammami-Hauasli N, Raghunath M, Bruckner-Tuderman L. Two forms of collagen XVII in keratinocytes: A full-length transmembrane protein and a soluble ectodomain. J Biol Chem. 1998; 273:25937–25943.
   PubMed Web of Science ®Google Scholar
• Hirako Y, Nishizawa Y, Sitaru C, Opitz A, Marcus K, Meyer HE, Butt E, Owaribe K, Zillikens D. The 97-kDa (LABD97) and 120-kDa (LAD-1) fragments of bullous pemphigoid antigen 180/type XVII collagen have different N-termini. J Invest Dermatol. 2003; 121:1554–1556.
   PubMed Web of Science ®Google Scholar
• Franzke CW, Bruckner-Tuderman L, Blobel CP. Shedding of collagen XVII/BP180 in skin depends on both ADAM10 and ADAM9. J Biol Chem. 2009; 284:23386–23396.
   PubMed Web of Science ®Google Scholar
• Hofmann SC, Voith U, Schönau V, Sorokin L, Bruckner-Tuderman L, Franzke CW. Plasmin plays a role in the in vitro generation of the linear IgA dermatosis antigen LADB97. J Invest Dermatol. 2009; 129:1730–1739.
   PubMed Web of Science ®Google Scholar
• Zillikens D, Herzele K, Georgi M, Schmidt E, Chimanovitch I, Schumann H, Mascaro JMJr, Diaz LA, Bruckner-Tuderman L, Bröcker EB, Giudice GJ. Autoantibodies in a subgroup of patients with linear IgA disease react with the NC16A domain of BP180. J Invest Dermatol. 1999a; 113:947–953.
   PubMed Web of Science ®Google Scholar
• Zambruno G, Manca V, Kanitakis J, Cozzani E, Nicolas JF, Giannetti A. Linear IgA bullous dermatosis with autoantibodies to a 290 kDa antigen of anchoring fibrils. J Am Acad Dermatol. 1994; 31:884–888.
   PubMed Web of Science ®Google Scholar
• Ghohestani RF, Nicolas JF, Kanitakis J, Claudy A. Linear IgA bullous dermatosis with IgA antibodies exclusively directed against the 180- or 230-kDa epidermal antigens. J Invest Dermatol. 1997; 108:854–858.
   PubMed Web of Science ®Google Scholar
• Natsuga K, Nishie W, Shinkuma S, Moriuchi R, Shibata M, Nishimura M, Hashimoto T, Shimizu H. Circulating IgA and IgE autoantibodies in antilaminin-332 mucous membrane pemphigoid. Br J Dermatol. 2010; 162:513–517.
   PubMed Web of Science ®Google Scholar
• Kromminga A, Scheckenbach C, Georgi M, Hagel C, Arndt R, Christophers E, Bröcker EB, Zillikens D. Patients with bullous pemphigoid and linear IgA disease show a dual IgA and IgG autoimmune response to BP180. J Autoimmun. 2000; 15:293–300.
   PubMed Web of Science ®Google Scholar
• Mulyowa GK, Jaeger G, Kabakyenga J, Bröcker EB, Zillikens D, Schmidt E. Autoimmune subepidermal blistering diseases in Uganda: Correlation of autoantibody class with age of patients. Int J Dermatol. 2006; 45:1047–1052.
   PubMed Web of Science ®Google Scholar
• 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.
   PubMedGoogle Scholar
• Guide SV, Marinkovich MP. Linear IgA bullous dermatosis. Clin Dermatol. 2001; 19:719–727.
   PubMed Web of Science ®Google Scholar
• Kasperkiewicz M, Meier M, Zillikens D, Schmidt E. Linear IgA disease: Successful application of immunoadsorption and review of the literature. Dermatology. 2010; 220:259–263.
   PubMed Web of Science ®Google Scholar
• Willsteed E, Bhogal BS, Das AK, Wojnarowska F, Black MM, McKee PH. Lichen planus pemphigoides: A clinicopathological study of nine cases. Histopathology. 1991; 19:147–154.
   PubMed Web of Science ®Google Scholar
• Davis AL, Bhogal BS, Whitehead P, Frith P, Murdoch ME, Leigh IM, Wojnarowska F. Lichen planus pemphigoides: Its relationship to bullous pemphigoid. Br J Dermatol. 1991; 125:263–271.
   PubMed Web of Science ®Google Scholar
• Bouloc A, Vignon-Pennamen MD, Caux F, Teillac D, Wechsler J, Heller M, Lebbé C, Flageul B, Morel P, Dubertret L, Prost C. Lichen planus pemphigoides is a heterogeneous disease: A report of five cases studied by immunoelectron microscopy. Br J Dermatol. 1998; 138:972–980.
   PubMed Web of Science ®Google Scholar
• Zillikens D, Caux F, Mascaro JM, Wesselmann U, Schmidt E, Prost C, Callen JP, Bröcker EB, Diaz LA, Giudice GJ. Autoantibodies in lichen planus pemphigoides react with a novel epitope within the C-terminal NC16A domain of BP180. J Invest Dermatol. 1999b; 113:117–121.
   PubMed Web of Science ®Google Scholar
• Zillikens D, Kawahara Y, Ishiko A, Shimizu H, Mayer J, Rank CV, Liu Z, Giudice GJ, Tran HH, Marinkovich MP, Brocker EB, Hashimoto T. A novel subepidermal blistering disease with autoantibodies to a 200-kDa antigen of the basement membrane zone. J Invest Dermatol. 1996; 106:1333–1338.
   PubMed Web of Science ®Google Scholar
• Dainichi T, Kurono S, Ohyama B, Ishii N, Sanzen N, Hayashi M, Shimono C, Taniguchi Y, Koga H, Karashima T, Yasumoto S, Zillikens D, Sekiguchi K, Hashimoto T. Anti-laminin gamma-1 pemphigoid. Proc Natl Acad Sci U S A. 2009; 106:2800–2805.
   PubMed Web of Science ®Google Scholar
• Groth S, Recke A, Vafia K, Ludwig RJ, Hashimoto T, Zillikens D, Schmidt E. Development of a simple enzyme-linked immunosorbent assay for the detection of autoantibodies in anti-p200 pemphigoid. Br J Dermatol. 2011; 164:76–82.
   PubMed Web of Science ®Google Scholar
• Timpl R, Rohde H, Robey PG, Rennard SI, Foidart JM, Martin GR. Laminin — A glycoprotein from basement membranes. J Biol Chem. 1979; 254:9933–9937.
   PubMed Web of Science ®Google Scholar
• Dainichi T, Koga H, Tsuji T, Ishii N, Ohyama B, Ueda A, Natsuaki Y, Karashima T, Nakama T, Yasumoto S, Zillikens D, Hashimoto T. From anti-p200 pemphigoid to anti-laminin gamma1 pemphigoid. J Dermatol. 2010; 37:231–238.
   PubMed Web of Science ®Google Scholar
• Iwata H, Hiramitsu Y, Aoyama Y, Kitajima Y. A case of anti-p200 pemphigoid: Evidence for a different pathway in neutrophil recruitment compared with bullous pemphigoid. Br J Dermatol. 2009; 160:462–464.
   PubMed Web of Science ®Google Scholar
• Dilling A, Rose C, Hashimoto T, Zillikens D, Shimanovich I. Anti-p200 pemphigoid: A novel autoimmune subepidermal blistering disease. J Dermatol. 2007; 34:1–8.
   PubMed Web of Science ®Google Scholar
• Rose C, Weyers W, Denisjuk N, Hillen U, Zillikens D, Shimanovich I. Histopathology of anti-p200 pemphigoid. Am J Dermatopathol. 2007; 29:119–124.
   PubMed Web of Science ®Google Scholar
• Kasperkiewicz M, Hoppe U, Zillikens D, Schmidt E. Relapse-associated autoantibodies to BP180 in a patient with anti-p200 pemphigoid. Clin Exp Dermatol. 2010; 35:614.
   PubMed Web of Science ®Google Scholar